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Sample records for acromegaly cushing disease

  1. Double, synchronous pituitary adenomas causing acromegaly and Cushing's disease. A case report and review of literature.

    Science.gov (United States)

    Zieliński, Grzegorz; Maksymowicz, Maria; Podgórski, Jan; Olszewski, Włodzimierz T

    2013-06-01

    Double pituitary adenomas are very rare and present up to 1 % of pituitary adenomas in unselected autopsy series and up to 2 % in large surgical series. We report a case of a 47-year-old man presented slight clinical features of acromegaly with 2 years duration. Endocrine evaluation confirmed active acromegaly and revealed adrenocorticotropin hormone-dependent hypercortisolemia. Preoperative magnetic resonance imaging of the pituitary demonstrated clearly separated double microadenomas with different intensity. The patient underwent transsphenoidal surgery and both tumors were completely removed and were fixed separately. The histological and ultrastructural examination confirmed coincidence of the double, clearly separated pituitary adenomas in one gland. Postoperative function of the hypothalamo-hypophyseal axis was normalized. We conclude from this case and a literature review that double endocrinologically active pituitary adenomas leading to acromegaly and Cushing's disease may occur. Additionally, a review of the literature regarding multiple pituitary adenomas has also been performed.

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

    Directory of Open Access Journals (Sweden)

    Bonert Vivien S

    2010-05-01

    Full Text Available Abstract Pituitary adenomas are associated with a variety of clinical manifestations resulting from excessive hormone secretion and tumor mass effects, and require a multidisciplinary management approach. This article discusses the treatment modalities for the management of patients with a prolactinoma, Cushing's disease and acromegaly, and summarizes the options for medical therapy in these patients. First-line treatment of prolactinomas is pharmacotherapy with dopamine agonists; recent reports of cardiac valve abnormalities associated with this class of medication in Parkinson's disease has prompted study in hyperprolactinemic populations. Patients with resistance to dopamine agonists may require other treatment. First-line treatment of Cushing's disease is pituitary surgery by a surgeon with experience in this condition. Current medical options for Cushing's disease block adrenal cortisol production, but do not treat the underlying disease. Pituitary-directed medical therapies are now being explored. In several small studies, the dopamine agonist cabergoline normalized urinary free cortisol in some patients. The multi-receptor targeted somatostatin analogue pasireotide (SOM230 shows promise as a pituitary-directed medical therapy in Cushing's disease; further studies will determine its efficacy and safety. Radiation therapy, with medical adrenal blockade while awaiting the effects of radiation, and bilateral adrenalectomy remain standard treatment options for patients not cured with pituitary surgery. In patients with acromegaly, surgery remains the first-line treatment option when the tumor is likely to be completely resected, or for debulking, especially when the tumor is compressing neurovisual structures. Primary therapy with somatostatin analogues has been used in some patients with large extrasellar tumors not amenable to surgical cure, patients at high surgical risk and patients who decline surgery. Pegvisomant is indicated in patients

  3. Hyperglycemia induced by pasireotide in patients with Cushing's disease or acromegaly.

    Science.gov (United States)

    Silverstein, Julie M

    2016-10-01

    Cushing's disease (CD) and acromegaly are characterized by excessive hormone secretion resulting in comorbidities such as impaired glucose metabolism, diabetes and hypertension. Pasireotide is a new-generation, multireceptor-targeted somatostatin receptor ligand approved for CD (subcutaneous [SC] injection formulation) and acromegaly (long-acting release [LAR] formulation). In clinical studies of pasireotide, hyperglycemia-related adverse events (AEs) were frequently observed. This review highlights differences in reported rates of hyperglycemia in pasireotide trials and discusses risk factors for and management of pasireotide-associated hyperglycemia. Clinical trials evaluating pasireotide in patients with CD or acromegaly were reviewed. The frequency of hyperglycemia-related AEs was lower in patients with acromegaly treated with pasireotide LAR (57.3-67.0 %) than in patients with CD treated with pasireotide SC (68.4-73.0 %). Fewer patients with acromegaly treated with pasireotide LAR discontinued therapy because of hyperglycemia-related AEs (Colao et al. in J Clin Endocrinol Metab 99(3):791-799, 2014, 3.4 %; Gadelha et al. in Lancet Diabetes Endocrinol 2(11):875-884, 2014, 4.0 %) than did patients with CD treated with pasireotide SC (Boscaro et al. in Pituitary 17(4):320-326, 2014, 5.3 %; Colao et al. in N Engl J Med 366(10):914-924, 2012, 6.0 %). Hyperglycemia-related AEs occurred in 40.0 % of patients with acromegaly treated with pasireotide SC, and 10.0 % discontinued treatment because of hyperglycemia. Ongoing studies evaluating pasireotide LAR in patients with CD and management of pasireotide-induced hyperglycemia in patients with CD or acromegaly (ClinicalTrials.gov identifiers NCT01374906 and NCT02060383, respectively) will address these key safety issues. Disease pathophysiology, drug formulation, and physician experience potentially influence the differences in reported rates of pasireotide-induced hyperglycemia in CD and acromegaly

  4. Pituitary tumor with gigantism, acromegaly and preclinical Cushing's disease diagnosed from the 10th row.

    Science.gov (United States)

    Tourtelot, John B; Vesely, David L

    2013-08-01

    A 7'3" basketball player was noted to have 2 to 3 times thicker tissue in his hands than 6'10" players by an endocrinologist sitting 10 rows above the player in a basketball arena. This led to the diagnosis of pituitary gigantism where the history revealed that he was 7'3" at 15 years of age. At age 19 when the acryl enlargement was noted, a diagnostic workup revealed elevated growth hormones and insulin-like growth factor 1 (IGF-1) with a 2 × 1.3 cm pituitary tumor. His history suggested that his epiphyseal plates had closed at age 15, and because he continued to produce IGF-1, he now has acromegaly. His elevated adrenocorticotropic hormone (ACTH) before surgery suggests that he also had preclinical Cushing's disease. After pituitary transsphenoidal surgery, all acryl enlargement in hands and ligaments disappeared. His growth hormone, IGF-1 and ACTH returned to normal 2 weeks after surgery.

  5. Advancing Treatment of Pituitary Adenomas through Targeted Molecular Therapies: The Acromegaly and Cushing Disease Paradigms

    Science.gov (United States)

    Mooney, Michael A.; Simon, Elias D.; Little, Andrew S.

    2016-01-01

    The current treatment of pituitary adenomas requires a balance of conservative management, surgical resection, and in select tumor types, molecular therapy. Acromegaly treatment is an evolving field where our understanding of molecular targets and drug therapies has improved treatment options for patients with excess growth hormone levels. We highlight the use of molecular therapies in this disease process and advances in this field, which may represent a paradigm shift for the future of pituitary adenoma treatment. PMID:27517036

  6. Advancing Treatment of Pituitary Adenomas through Targeted Molecular Therapies: The Acromegaly & Cushing Disease Paradigms

    Directory of Open Access Journals (Sweden)

    Michael Anthony Mooney

    2016-07-01

    Full Text Available The current treatment of pituitary adenomas requires a balance of conservative management, surgical resection, and in select tumor types, molecular therapy. Acromegaly treatment is an evolving field where our understanding of molecular targets and drug therapies has improved treatment options for patients with excess growth hormone levels. We highlight the use of molecular therapies in this disease process and advances in this field, which may represent a paradigm shift for the future of pituitary adenoma treatment.

  7. Dramatic Improvement of Diabetes Mellitus Following the Treatment of Coexisting Acromegaly and Cushing's Syndrome.

    Science.gov (United States)

    Kim, Soo Kyoung; Kim, Bo Ra; Kim, Kyongyoung; Kim, Sungsu; Jung, Jung Hwa; Hahm, Jong Ryeal; Jung, Jaehoon

    2015-01-01

    Endocrine diseases are frequently accompanied by diabetes mellitus and treatment of an underlying endocrine disease often improves glucose control. The co-occurrence of acromegaly and Cushing's syndrome is extremely rare. We herein describe a patient who showed a dramatic improvement in glucose control following treatment for co-existing acromegaly and Cushing's syndrome. An adrenal mass was incidentally discovered during a routine evaluation of a 56-year-old woman who was subsequently diagnosed with acromegaly and a unilateral cortisol-producing adrenal adenoma. Her blood glucose was poorly controlled despite receiving high-dose insulin therapy. After undergoing adrenalectomy for Cushing's syndrome, her insulin dosage was decreased by almost 50%. The insulin treatment was discontinued following the treatment of acromegaly.

  8. Cushing, acromegaly, GH deficiency and tendons

    OpenAIRE

    2014-01-01

    Cushing’s syndrome, induced by an endogenous or exogenous cortisol excess, and acromegaly, the clinical syndrome caused by growth hormone (GH) excess in adulthood, as well as the disease induced by GH deficiency (GHD), represent perfect models for the evaluation of the effects induced by chronic exposure in vivo, respectively, to cortisol and GH/IGF-1 excess or deficiency on the complex structure of the tendons as well as on the related post-traumatic repair mechanism. Although the literature...

  9. Harvey Cushing: Cushing's disease.

    Science.gov (United States)

    Ellis, Harold

    2012-09-01

    In 1932 Harvey Cushing published an article entitled 'Basophil adenomas of the pituitary and their clinical manifestations'. These are rare tumours of the anterior pituitary gland, at the base of the brain, with striking manifestations, which include adiposity of the face and trunk, but not the limbs, weakness, fatigue, abdominal striae, facial hair, high blood pressure and raised blood sugar. Although I spent two years in an army neurosurgical unit, I never saw a case. More frequently, the same clinical picture is produced by a benign adenoma of the suprarenal cortex, but today by far the commonest cause of this condition is seen in the patient receiving high dosage of cortisone, when we talk about the person exhibiting 'Cushingoid features'. Most readers of this journal will have seen such cases.

  10. Treating Cushing's Disease in Dogs

    Science.gov (United States)

    ... Devices Radiation-Emitting Products Vaccines, Blood & Biologics Animal & Veterinary Cosmetics Tobacco Products ... Español On this page: Two Common Types Symptoms of Cushing's Disease Treating Cushing's Disease Your 9- ...

  11. On the shoulders of giants: Harvey Cushing's experience with acromegaly and gigantism at the Johns Hopkins Hospital, 1896-1912.

    NARCIS (Netherlands)

    Pendleton, C.; Adams, H.; Salvatori, R.; Wand, G.; Quinones-Hinojosa, A.

    2011-01-01

    A review of Dr. Cushing's surgical cases at Johns Hopkins Hospital revealed new information about his early operative experience with acromegaly. Although in 1912 Cushing published selective case studies regarding this work, a review of all his operations for acromegaly during his early years has ne

  12. On the shoulders of giants: Harvey Cushing's experience with acromegaly and gigantism at the Johns Hopkins Hospital, 1896-1912.

    NARCIS (Netherlands)

    Pendleton, C.; Adams, H.; Salvatori, R.; Wand, G.; Quinones-Hinojosa, A.

    2011-01-01

    A review of Dr. Cushing's surgical cases at Johns Hopkins Hospital revealed new information about his early operative experience with acromegaly. Although in 1912 Cushing published selective case studies regarding this work, a review of all his operations for acromegaly during his early years has ne

  13. Fat deposition in the cavernous sinus in Cushing disease

    Energy Technology Data Exchange (ETDEWEB)

    Bachow, T.B.; Hesselink, J.R.; Aaron, J.O.; Davis, K.R.; Taveras, J.M.

    1984-10-01

    Fat density in the cavernous sinus on computed tomography (CT) is described in 6 out of 16 (37.5%) patients with Cushing disease. This finding may aid in making a specific diagnosis in patients with a pituitary mass. It was not seen in 30 random CT studies of the sella; however, supra seller fat was incidentally noted in the patient with acromegaly.

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

    LENUS (Irish Health Repository)

    Noctor, E

    2015-06-01

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

  15. On the shoulders of giants: Harvey Cushing's experience with acromegaly and gigantism at the Johns Hopkins Hospital, 1896-1912.

    Science.gov (United States)

    Pendleton, Courtney; Adams, Hadie; Salvatori, Roberto; Wand, Gary; Quiñones-Hinojosa, Alfredo

    2011-03-01

    A review of Dr. Cushing's surgical cases at Johns Hopkins Hospital revealed new information about his early operative experience with acromegaly. Although in 1912 Cushing published selective case studies regarding this work, a review of all his operations for acromegaly during his early years has never been reported. We uncovered 37 patients who Cushing treated with surgical intervention directed at the pituitary gland. Of these, nine patients who presented with symptoms of acromegaly, and one with symptoms of gigantism were selected for further review. Two patients underwent transfrontal 'omega incision' approaches, and the remaining eight underwent transsphenoidal approaches. Of the 10 patients, 6 were male. The mean age was 38.0 years. The mean hospital stay was 39.4 days. There was one inpatient death during primary interventions (10%) and three patients were deceased at the time of last follow-up (33%). The mean time to death, calculated from the date of the primary surgical intervention, and including inpatient and outpatient deaths, was 11.3 months. The mean time to last follow-up, calculated from the day of discharge, was 59.3 months. At the time of last follow-up, two patients reported resolution of headache; four patients reported continued visual deficits, and two patients reported ongoing changes in mental status. This review analyzes the outcomes for 10 patients who underwent surgical intervention for acromegaly or gigantism, and offers an explanation for Cushing's transition from the transfrontal "omega incision" to the transsphenoidal approach while practicing at the Johns Hopkins Hospital.

  16. Unmasking sarcoidosis following surgery for Cushing disease

    DEFF Research Database (Denmark)

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

  17. Unmasking sarcoidosis following surgery for Cushing disease

    DEFF Research Database (Denmark)

    Diernæs, Jon Erik; Bygum, Anette; Poulsen, Per L

    2016-01-01

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

  18. Acromegaly.

    Science.gov (United States)

    Nabarro, J D

    1987-04-01

    A personal series of 256 cases of acromegaly/gigantism seen over a 20-year period from 1963 is described. The insidious nature of the condition resulted in delay in diagnosis which was often made by a doctor when seeing the patient for an unrelated problem. Other features which commonly led to the diagnosis being made were headache, change in appearance, carpal tunnel syndrome, amenorrhoea and diabetes. The Hardy system for grading the radiological appearance of the pituitary tumour was used. Widely invasive tumours were not common but tended to occur in patients with younger age of onset and high GH levels. The occurrence of various symptoms and clinical features was noted and the changes resulting from reducing the GH level to normal. The incidence of hypertension, but not of coronary artery disease, is increased and the blood pressure may be reduced following successful treatment. The effects on the upper and lower respiratory tract are reported as well as sleep apnoea and problems associated with anaesthesia. Skin manifestations included sweating, pigmented skin tags, acanthosis nigricans and cutis verticis gyrata. In the skeletal system the incidence of kyphoscoliosis and osteoarthritis especially of the hip is reported: the question of hip replacement is discussed. Diabetes mellitus disappeared in most cases if the acromegaly was cured. In men but not in women the incidence of colloid nodular goitre was increased as was hyperthyroidism in middle-aged women. In two patients a parathyroid adenoma was present: hypercalcaemia was present in five additional patients, but the cause was not determined. The common occurrence of amenorrhoea in the younger women was noted, it was not always associated with hyperprolactinaemia, and often responded to successful treatment of the acromegaly. The association of acromegaly with hirsutism and galactorrhoea is confirmed. The incidence of impotence and loss of libid in the men is discussed: in a proportion of those in whom the

  19. Acromegaly

    Directory of Open Access Journals (Sweden)

    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

  20. Role of adrenalectomy in recurrent Cushing's disease

    Institute of Scientific and Technical Information of China (English)

    DING Xue-fei; LI Han-zhong; YAN Wei-gang; GAO Ying; LI Xiao-qiang

    2010-01-01

    Background Cushing's disease is a pituitary-dependent type of Cushing's syndrome. Treatment consists of pituitary surgery or radiotherapy, but the recurrence rate at 10 years is as high as 40%. Adrenalectomy is considered an effective treatment to refractory Cushing's disease. The objective of this study was to examine the efficacy of laparoscopic adrenalectomy and open adrenalectomy in Cushing's disease, focusing on reversing the sequelae of hypercortisolism and improving patients' quality of life.Methods Forty-three patients (29 women, 14 men) with recurrent Cushing's disease after transsphenoidal operation underwent laparoscopic (n=32) or open (n=11) adrenalectomy from 2000 to 2008. Surgical results were evaluated for all the 43 patients. Patients completed a follow-up survey, including the short-form 36-item (SF-36) health survey. Results All the 43 patients achieved clinical reversal of hypercortisolism after adrenalectomy. Time to symptom resolution varied from a few weeks to up to 3 years. Most physical changes had resolved by a mean of 8 months after surgery. These conditions were not significantly different between the laparoscopy and open groups. Median length of hospital stay was shorter in the laparoscopy group (4 vs. 9 days; P200 ng/ml and 6 (27%) had clinical Nelson syndrome. Four patients died by 75 months after surgery. Using SF-36, 30 (88%) patients reported they felt their health status was good to excellent compared with 1 year before adrenalectomy; however, they showed significantly lower scores in all the 8 SF-36 parameters compared with the general population. No significant difference emerged in SF-36 scores between the laparoscopy and open groups.Conclusions Adrenalectomy showed high survival and clinical benefits in recurrent Cushing's disease patients. Despite patient-reported improvement in health after adrenalectomy, patients continue to experience poor health status compared with the general population.

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

    Energy Technology Data Exchange (ETDEWEB)

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

    1994-12-01

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

  2. Pituitary magnetic resonance imaging in Cushing's disease.

    Science.gov (United States)

    Vitale, Giovanni; Tortora, Fabio; Baldelli, Roberto; Cocchiara, Francesco; Paragliola, Rosa Maria; Sbardella, Emilia; Simeoli, Chiara; Caranci, Ferdinando; Pivonello, Rosario; Colao, Annamaria

    2017-03-01

    Adrenocorticotropin-secreting pituitary tumor represents about 10 % of pituitary adenomas and at the time of diagnosis most of them are microadenomas. Transsphenoidal surgery is the first-line treatment of Cushing's disease and accurate localization of the tumor within the gland is essential for selectively removing the lesion and preserving normal pituitary function. Magnetic resonance imaging is the best imaging modality for the detection of pituitary tumors, but adrenocorticotropin-secreting pituitary microadenomas are not correctly identified in 30-50 % of cases, because of their size, location, and enhancing characteristics. Several recent studies were performed with the purpose of better localizing the adrenocorticotropin-secreting microadenomas through the use in magnetic resonance imaging of specific sequences, reduced contrast medium dose and high-field technology. Therefore, an improved imaging technique for pituitary disease is mandatory in the suspect of Cushing's disease. The aims of this paper are to present an overview of pituitary magnetic resonance imaging in the diagnosis of Cushing's disease and to provide a magnetic resonance imaging protocol to be followed in case of suspicion adrenocorticotropin-secreting pituitary adenoma.

  3. Unmasking sarcoidosis following surgery for Cushing disease

    Science.gov (United States)

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

    2016-01-01

    ABSTRACT 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

  4. Serratia marcescens osteomyelitis in Cushing's disease.

    Science.gov (United States)

    Martins, Hugo F G; Raposo, Alexandra; Baptista, Isabel; Almeida, Julio

    2015-11-30

    We report a case of a 46-year-old man with fever, hypotension and arthralgias of the ankles and knees after brain surgery for a pituitary tumour causing Cushing's disease. Blood and urine cultures isolated Serratia marcescens; antibiotic susceptibility testing showed sensitivity to piperacillin-tazobactan and ciprofloxacin. Articular MRI showed inflammation and necrosis of both knees and ankles, and left hip and right elbow (compatible with osteomyelitis). Culture of an ankle abscess on the ankle joint was positive for Serratia marcescens. Bone scintigraphy confirmed osteomyelitic lesions. Medical treatment included antibiotics and strong opioid therapy for 14 weeks. The patient was discharged clinically improved maintaining ciprofloxacin for 24 additional weeks based on clinical and analytic recovery.

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

    Energy Technology Data Exchange (ETDEWEB)

    Maeder, P. [Dept. of Radiology, Centre Hospitalier Universitaire Vaudois, Lausanne (Switzerland); Gudinchet, F. [Dept. of Radiology, Centre Hospitalier Universitaire Vaudois, Lausanne (Switzerland); Rillet, B. [Dept. of Neurosurgery, Centre Hospitalier Universitaire Vaudois, Lausanne (Switzerland); Theintz, G. [Dept. of Paediatrics, Centre Hospitalier Universitaire Vaudois, Lausanne (Switzerland); Meuli, R. [Dept. of Radiology, Centre Hospitalier Universitaire Vaudois, Lausanne (Switzerland)

    1996-01-01

    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.)

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

    Science.gov (United States)

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

    2017-03-01

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

  7. Paradoxical response to dexamethasone and spontaneous hypocortisolism in Cushing's disease.

    Science.gov (United States)

    Lila, Anurag R; Sarathi, Vijaya; Bandgar, Tushar R; Shah, Nalini S

    2013-01-29

    Paradoxical response to dexamethasone and spontaneous development of hypocortisolism are rare features of Cushing's disease. We report a 13-year-old boy with Cushing's disease owing to a pituitary macroadenoma. On initial evaluation, he had partial suppression of serum cortisol by dexamethasone. He developed transient hypocortisolism after first adenomectomy, but the disease recurred after 1 year. Repeat evaluation showed recurrent hypercortisolism and paradoxical response to dexamethasone. He underwent second surgery and, postoperatively, hypercostisolism persisted even after 2 years of surgery. Repeat evaluations after 8 years of second surgery revealed persistent hypocortisolism despite residual tumour of same size and similar plasma adrenocorticotropic hormone (ACTH) levels. We have also shown that the paradoxical increase in serum cortisol was preceded by a paradoxical increase in ACTH. The paradoxical response persisted despite hypocortisolism. This patient with Cushing's disease had two very rare features: paradoxical response to dexamethasone and spontaneous development of hypocortisolism.

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

    Energy Technology Data Exchange (ETDEWEB)

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

    2003-02-01

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

  9. MANAGEMENT OF ENDOCRINE DISEASE: Acromegaly and pregnancy: a contemporary review.

    Science.gov (United States)

    Abucham, Julio; Bronstein, Marcello D; Dias, Monike L

    2017-07-01

    Although fertility is frequently impaired in women with acromegaly, pregnancy is apparently becoming more common due to improvement in acromegaly treatment as well as in fertility therapy. As a result, several studies on pregnancy in patients with acromegaly have been published in recent years adding new and relevant information to the preexisting literature. Also, new GH assays with selective specificities and the knowledge of the expression of the various GH genes have allowed a better understanding of somatotrophic axis function during pregnancy. In this review, we show that pregnancy in women with acromegaly is generally safe, usually with tumoral and hormonal stability. Although the paucity of data limits evidence-based recommendations for preconception counseling and pregnancy surveillance, controlling tumor size and hormonal activity before pregnancy is highly recommended to ensure better outcomes, and surgical control should be attempted when feasible. Treatment interruption at pregnancy confirmation has also proven to be safe, as drugs are not formally allowed to be used during pregnancy. Drug exposure (somatostatin analogs) during early or whole pregnancy might increase the chance of a lower birth weight. Aggressive disease is uncommon and may urge individual decisions such as surgery or drug treatment during pregnancy or lactation. © 2017 European Society of Endocrinology.

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

    Directory of Open Access Journals (Sweden)

    So-Young Kim

    2015-06-01

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

  11. Acromegaly

    Science.gov (United States)

    ... disease: Colon polyps Excess hair growth in females ( hirsutism ) Type 2 diabetes Weight gain (unintentional) Exams and ... rest of the pituitary gland is working normally. Treatment Surgery to remove the pituitary tumor that is ...

  12. CT pulmonary densitovolumetry in patients with acromegaly: a comparison between active disease and controlled disease

    Science.gov (United States)

    Camilo, Gustavo B; Carvalho, Alysson R S; Machado, Dequitier C; Mogami, Roberto; Melo, Pedro L

    2015-01-01

    Objective: Our purpose was to compare the findings of CT pulmonary densitovolumetry and pulmonary function in patients with active acromegaly and controlled acromegaly and, secondarily, to correlate these findings. Methods: 11 patients with active acromegaly, 18 patients with controlled acromegaly and 17 control subjects, all non-smokers, underwent quantification of lung volume using multidetector CT (Q-MDCT) and pulmonary function tests. Results: Patients with active acromegaly had larger total lung mass (TLM) values than the controls and larger amounts of non-aerated compartments than the other two groups. Patients with active acromegaly also had larger amounts of poorly aerated compartments than the other two groups, a difference that was observed in both total lung volume (TLV) and TLM. TLV as measured by inspiratory Q-MDCT correlated significantly with total lung capacity, whereas TLV measured using expiratory Q-MDCT correlated significantly with functional residual capacity. Conclusion: Patients with active acromegaly have more lung mass and larger amounts of non-aerated and poorly aerated compartments. There is a relationship between the findings of CT pulmonary densitovolumetry and pulmonary function test parameters. Advances in knowledge: Although the nature of our results demands further investigation, our data suggest that both CT pulmonary densitovolumetry and pulmonary function tests can be used as useful tools for patients with acromegaly by assisting in the prediction of disease activity. PMID:26246281

  13. CT pulmonary densitovolumetry in patients with acromegaly: a comparison between active disease and controlled disease.

    Science.gov (United States)

    Camilo, Gustavo B; Carvalho, Alysson R S; Machado, Dequitier C; Mogami, Roberto; Melo, Pedro L; Lopes, Agnaldo J

    2015-10-01

    Our purpose was to compare the findings of CT pulmonary densitovolumetry and pulmonary function in patients with active acromegaly and controlled acromegaly and, secondarily, to correlate these findings. 11 patients with active acromegaly, 18 patients with controlled acromegaly and 17 control subjects, all non-smokers, underwent quantification of lung volume using multidetector CT (Q-MDCT) and pulmonary function tests. Patients with active acromegaly had larger total lung mass (TLM) values than the controls and larger amounts of non-aerated compartments than the other two groups. Patients with active acromegaly also had larger amounts of poorly aerated compartments than the other two groups, a difference that was observed in both total lung volume (TLV) and TLM. TLV as measured by inspiratory Q-MDCT correlated significantly with total lung capacity, whereas TLV measured using expiratory Q-MDCT correlated significantly with functional residual capacity. Patients with active acromegaly have more lung mass and larger amounts of non-aerated and poorly aerated compartments. There is a relationship between the findings of CT pulmonary densitovolumetry and pulmonary function test parameters. Although the nature of our results demands further investigation, our data suggest that both CT pulmonary densitovolumetry and pulmonary function tests can be used as useful tools for patients with acromegaly by assisting in the prediction of disease activity.

  14. Low risk of coronary artery disease in patients with acromegaly.

    Science.gov (United States)

    Dos Santos Silva, Cintia Marques; Lima, G A B; Volschan, I C M; Gottlieb, I; Kasuki, L; Neto, L Vieira; Gadelha, M R

    2015-12-01

    The aims of this study are to determine the prevalence of coronary atherosclerosis in acromegalic patients and to investigate the relationship between the coronary artery calcium score (CS) and acromegaly status and clinical parameters [Framingham risk score (FRS)]. Fifty-six acromegalic patients and paired non-acromegalic volunteers were stratified according to the FRS into low-, intermediate-, and high-risk groups. CS was assessed using multidetector computed tomography. The patients were considered to have controlled or active acromegaly at the time they were submitted to evaluation. Sixty-six percent of acromegalic patients exhibited arterial hypertension, 36 % had diabetes mellitus, and 34 % had hypercholesterolemia. The median FRS and the median risk for cardiovascular event within the next 10 years were similar in the acromegalics and the controls. The median total CS and CS >75th percentile didn't differ significantly between these groups. In patients with controlled acromegaly, a low, intermediate, or high FRS risk was observed in 86, 14, and 0 %, respectively. In patients with active disease, a low, intermediate, or high FRS risk was verified in 94, 3, and 3 %, respectively, and differences between the controlled and active groups were not significant. Seventy-two percent of the patients had total CS = 0, and there were no differences between the controlled and active groups. The risk of coronary artery disease in acromegalic patients, determined according to FRS and CS, is low despite the high prevalence of metabolic abnormalities.

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

    Directory of Open Access Journals (Sweden)

    Stephanie Smooke Praw

    2009-10-01

    Full Text Available Stephanie Smooke Praw1, Anthony P Heaney1,21Department of Medicine, 2Department of Neurosurgery, David Geffen School of Medicine at UCLA, Los Angeles, CA, USAAbstract: Cushing's disease, due to pituitary adrenocorticotropic hormone (ACTH hypersecretion, is the most common etiology of spontaneous excess cortisol production. The majority of pituitary tumors causing Cushing's disease measure <1 cm and the excess morbidity associated with these tumors is mostly due to the effects of elevated, nonsuppressible, ACTH levels leading to adrenal steroid hypersecretion. Elevated circulating cortisol levels lead to abnormal fat deposition, hypertension, diabetes, coronary artery disease, osteoporosis, muscle weakness and psychological disturbances. At experienced centers, initial surgical remission rate via transnasal, transphenoidal resection approaches 80% for tumors less than 1 cm, but may be as low as 30% for larger lesions and long-term recurrence in all groups approaches 25%. Residual disease may be managed with more radical surgery, pituitary-directed radiation, bilateral adrenalectomy, or medical therapy. This paper addresses current and novel therapies in various stages of development for Cushing’s disease.Keywords: Cushing's disease, treatment, pasireotide, PPAR-γ, 11 β-hydroxysteroid dehydrogenase inhibitors, dopamine agonists

  16. Gamma knife radiosurgery for Cushing's disease and Nelson's syndrome.

    Science.gov (United States)

    Marek, Josef; Ježková, Jana; Hána, Václav; Kršek, Michal; Liščák, Roman; Vladyka, Vilibald; Pecen, Ladislav

    2015-06-01

    This paper presents our 18 years of experience in treating ACTH secreting adenomas (Cushing's disease and Nelson's syndrome) using the Leksell gamma knife (LGK) irradiation. Twenty-six patients with Cushing's disease were followed-up after LGK irradiation for 48-216 months (median 78 months). Seventeen patients had undergone previous surgery, in nine patients LGK irradiation was the primary therapy. Furthermore, 14 patients with Nelson's syndrome were followed-up for 30-204 months (median 144 months). LGK treatment resulted in hormonal normalization in 80.7 % of patients with Cushing's disease. Time to normalization was 6-54 months (median 30 months). The volume of the adenoma decreased in 92.3% (in 30.7% disappeared completely). There was no recurrence of the disease. In all 14 patients with Nelson's syndrome ACTH levels decreased (in two patients fully normalized) their ACTH levels. When checked up 5-10 years after irradiation regrowth of the adenoma was only detected in one patient (9.1%), in 27.3% adenoma volume remained unchanged, in 45.4% adenoma volume decreased and in 18.2% adenoma completely disappeared. Hypopituitarism did not develop in any patient where the critical dose to the pituitary and distal infundibulum was respected. LGK radiation represents an effective and well-tolerated option for the treatment of patients with Cushing's disease after unsuccessful surgery and may be valuable even as a primary treatment in patients who are not suitable for, or refuse, surgery. In the case of Nelson's syndrome it is possible to impede tumorous growth and control the size of the adenoma in almost all patients.

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

    Directory of Open Access Journals (Sweden)

    Fatemeh Esfahanian

    2010-08-01

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

  18. Diagnostic Usefulness of 3 Tesla MRI of the Brain for Cushing Disease in a Child

    OpenAIRE

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

    2011-01-01

    It is sometimes difficult to confirm the location of a microadenoma in Cushing disease. Recently, we experienced an 11-yr-old female case of Cushing disease with hyperprolactinemia. She was referred to our hospital because of decrease of height velocity with body weight gain. On admission, she had typical symptoms of Cushing syndrome. Although no pituitary microadenomas were detected on 1.5 Tesla MRI of the brain, endocrinological examinations including IPS and CS sampling were consistent wit...

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

    Science.gov (United States)

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

    2015-09-01

    Measurement of the plasma adrenocorticotropic hormone (ACTH) level has been recommended as the first diagnostic test for differentiating between ACTH-independent Cushing syndrome (CS) and ACTH-dependent CS. When plasma ACTH values are inconclusive, a differential diagnosis of CS can be made based upon measurement of the serum dehydroepiandrosterone sulfate (DHEA-S) level and results of the high-dose dexamethasone suppression test (HDST). The aim of this study was to assess the utility of plasma ACTH to differentiate adrenal CS from Cushing' disease (CD) and compare it with that of the HDST results and serum DHEA-S level. We performed a retrospective, multicenter study from January 2000 to May 2012 involving 92 patients with endogenous CS. The levels of plasma ACTH, serum cortisol, 24-hour urine free cortisol (UFC) after the HDST, and serum DHEA-S were measured. Fifty-seven patients had adrenal CS and 35 patients had CD. The area under the curve of plasma ACTH, serum DHEA-S, percentage suppression of serum cortisol, and UFC after HDST were 0.954, 0.841, 0.950, and 0.997, respectively (all P<0.001). The cut-off values for plasma ACTH, percentage suppression of serum cortisol, and UFC after HDST were 5.3 pmol/L, 33.3%, and 61.6%, respectively. The sensitivity and specificity of plasma ACTH measurement were 84.2% and 94.3%, those of serum cortisol were 95.8% and 90.6%, and those of UFC after the HDST were 97.9% and 96.7%, respectively. Significant overlap in plasma ACTH levels was seen between patients with adrenal CS and those with CD. The HDST may be useful in differentiating between these forms of the disease, especially when the plasma ACTH level alone is not conclusive.

  20. Cushing's disease: establishing the diagnosis and management approach.

    Science.gov (United States)

    Varadhan, Lakshminarayanan; Aror, Amit; Walker, Adrian B; Varughese, George Iype

    2013-04-01

    A 64 year old lady, with a background history of type 2 diabetes mellitus and hypertension, presented with general deterioration of general health, poor glycemic control, difficulty in controlling blood pressure and difficulty in walking. She had past medical history of adenocarcinoma of the oesophagus, treated with surgery and subsequent chemotherapy. General examination revealed high blood glucose and blood pressure and a Cushingoid facies. Overnight dexamethasone suppression test and urinary free cortisol levels confirmed Cushing's syndrome and High dose dexamethasone suppression test showed partial suppression. CT scan of the abdomen showed bilateral hyperplasia of the adrenals with nodularity on the left side, raising the possibility of an adrenal adenoma. ACTH levels were elevated thereby ruling out autonomously functioning adrenal nodule, however increasing the possibility of ectopic ACTH secretion due to the previous medical history. MRI of the pituitary confirmed the presence of an adenoma, thereby pointing to the diagnosis of pituitary dependant Cushing's disease. The patient could not undergo further invasive investigation or surgery due to septicaemia. Medical management of Cushing's syndrome was resorted to in the interim with Ketoconazole, showing excellent response. This case depicts the need for a high index of suspicion for the diagnosis, the importance of organizing specific investigations in the appropriate order to arrive at a diagnosis and an effective management plan.

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

    Directory of Open Access Journals (Sweden)

    Eun Kyoung Lee

    2014-10-01

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

  2. Detecting acromegaly: screening for disease with a morphable model.

    Science.gov (United States)

    Learned-Miller, Erik; Lu, Qifeng; Paisley, Angela; Trainer, Peter; Blanz, Volker; Dedden, Katrin; Miller, Ralph

    2006-01-01

    Acromegaly is a rare disorder which affects about 50 of every million people. The disease typically causes swelling of the hands, feet, and face, and eventually permanent changes to areas such as the jaw, brow ridge, and cheek bones. The disease is often missed by physicians and progresses beyond where it might if it were identified and treated earlier. We consider a semi-automated approach to detecting acromegaly, using a novel combination of support vector machines (SVMs) and a morphable model. Our training set consists of 24 frontal photographs of acromegalic patients and 25 of disease-free subjects. We modelled each subject's face in an analysis-by-synthesis loop using the three-dimensional morphable face model of Blanz and Vetter. The model parameters capture many features of the 3D shape of the subject's head from just a single photograph, and are used directly for classification. We report encouraging results of a classifier built from the training set of real human subjects.

  3. Quality of life in patients after long-term biochemical cure of cushing's disease

    NARCIS (Netherlands)

    M.O. van Aken (Maarten); A.M. Pereira (Alberto); N.R. Biermasz; S.W. van Thiel (Sjoerd); H. Hoftijzer (Hendrieke); J.W. Smit (Johannes); F. Roelfsema (Ferdinand); S.W.J. Lamberts (Steven); J.A. Romijn (Johannes)

    2005-01-01

    textabstractTo evaluate the long-term impact of cured Cushing's disease on subjective well-being, we assessed quality of life by validated health-related questionnaires in 58 patients cured from Cushing's disease by transsphenoidal surgery (n = 58), some of whom received additional radiotherapy (n =

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

    Science.gov (United States)

    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. © 2016 European Society of Endocrinology.

  5. Magnetic resonance imaging and histology correlation in Cushing's disease.

    Science.gov (United States)

    Masopust, Václav; Netuka, David; Beneš, Vladimír; Májovský, Martin; Belšán, Tomáš; Bradáč, Ondřej; Hořínek, Daniel; Kosák, Mikuláš; Hána, Václav; Kršek, Michal

    We continuously look for new techniques to improve the radicality of resection and to eliminate the negative effects of surgery. One of the methods that has been implemented in the perioperative management of Cushing's disease was the combination of three magnetic resonance imaging (MRI) sequences: SE, SPGR and fSPGR. We enrolled 41 patients (11 males, 30 females) diagnosed with Cushing's disease. A 3D tumour model with a navigation console was developed using each SPGR, fSPGR and SE sequence. The largest model was then used. In all cases, a standard four-handed, bi-nostril endoscopic endonasal technique was used. Endocrinological follow-up evaluation using morning cortisol sampling was performed for 6-34 months in our study. In total, 36 patients (88%) were disease-free following surgery. Our results indicate we achieved 100% sensitivity of MR. Overall, the conformity of at least one donor site, as compared with the places designated on MR, was in 78% of patients. We searched the place of compliance in individual locations. There is a consensus in individual locations in 63 of the 123 cases (or 56%). The correlation gamma function at a 5% significance level was then 0.27. The combination of MR sequences (SE, SPGR, fSPGR), neuronavigation system and iMRI led to increased sensitivity of up to 100%. Specificity reached 56% in our study. We found a high success rate in surgical procedure in terms of the correlation between MR findings and histology, which leads to remission of Cushing's disease. Copyright © 2016. Published by Elsevier Urban & Partner Sp. z o.o.

  6. Treatment of Cushing disease: overview and recent findings

    Directory of Open Access Journals (Sweden)

    Tatiana Mancini

    2010-10-01

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

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

    LENUS (Irish Health Repository)

    Hassan-Smith, Zaki K

    2012-04-01

    Historically, Cushing\\'s disease (CD) was associated with a 5-yr survival of just 50%. Although advances in CD management have seen mortality rates improve, outcome from transsphenoidal surgery (TSS), the current first-line treatment, varies significantly between centers.

  8. FGFR4 polymorphic variants modulate phenotypic features of Cushing disease.

    Science.gov (United States)

    Nakano-Tateno, Tae; Tateno, Toru; Hlaing, Maw Maw; Zheng, Lei; Yoshimoto, Katsuhiko; Yamada, Shozo; Asa, Sylvia L; Ezzat, Shereen

    2014-04-01

    Cushing disease is a potentially lethal condition resulting from hormone excess, usually due to a small pituitary tumor that fails to respond to negative feedback inhibition. A minority of patients develop larger, more aggressive tumors of the same lineage but with modest hormone excess. Here we show that a common polymorphism in the fibroblast growth factor receptor 4 (FGFR4) transmembrane domain yields receptor isoforms with distinct properties that mediate these biological differences. Forced expression of the major FGFR4-G388 variant allele supports pY-signal transducer and activator of transcription (STAT3) responses. In contrast, expression of the minor FGFR4-R388 allele enhances STAT3 serine phosphorylation, driving cellular growth. In addition, FGFR4-R388 enhances glucocorticoid receptor phosphorylation and nuclear translocation. Consistent with these findings, glucocorticoid administration resulted in enhanced hormone negative feedback in mice with knock-in of the FGFR4 variant allele. Moreover, clinical data from patients with pituitary tumors revealed that those homozygous for the R388 allele have a higher frequency of silent corticotroph macroadenomas than FGFR4-G388 carriers, who were more likely to have small but hormonally active microadenomas. These findings demonstrate that the FGFR4 transmembrane polymorphic variants can modulate cellular growth and sensitivity to glucocorticoid hormone negative feedback through distinct STAT3 modifications of relevance to the human forms of Cushing disease.

  9. Cushing's Disease Presented by Reversible Dilated Cardiomyopathy

    OpenAIRE

    Berna İmge Aydoğan; Demet Menekşe Gerede; Asena Gökçay Canpolat; Murat Faik Erdoğan

    2015-01-01

    Introduction. Dilated cardiomyopathy is rarely reported among CS patients especially without hypertension and left ventricular hypertrophy. Materials and Methods. We hereby report a Cushing’s syndrome case presenting with dilated cardiomyopathy. Results. A 48-year-old female patient was admitted to our clinic with severe proximal myopathy and dilated cardiomyopathy without ventricular hypertrophy. Cushing’s disease was diagnosed and magnetic-resonance imaging of the pituitary gland revealed a...

  10. Sequelae to acromegaly: reversibility with treatment of the primary disease.

    Science.gov (United States)

    Lieberman, S A; Hoffman, A R

    1990-06-01

    Acromegalic patients suffer from a number of cardiovascular, metabolic, and rheumatologic problems, and they may also have an increased incidence of malignancy. We reviewed the literature concerning the reversibility of acromegalic complications. Hypertension, myocardial hypertrophy, left ventricular dysfunction and some rheumatologic abnormalities often continue despite successful treatment of the acromegaly. In contrast, glucose intolerance, soft tissue changes, and carpal tunnel syndrome usually resolve when the acromegaly is cured. Studies of the incidence and mortality of cancer in acromegaly are conflicting, but several suggest an increased incidence of colorectal cancer.

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

    DEFF Research Database (Denmark)

    Colao, Annamaria; Petersenn, Stephan; Newell-Price, John

    2012-01-01

    Cushing's disease is associated with high morbidity and mortality. Pasireotide, a potential therapy, has a unique, broad somatostatin-receptor-binding profile, with high binding affinity for somatostatin-receptor subtype 5....

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

    Science.gov (United States)

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

    2011-10-01

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

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

    Directory of Open Access Journals (Sweden)

    MARCELO MAIA PINHEIRO

    1999-09-01

    Full Text Available A doença de Cushing é rara em crianças e sua ocorrência em gêmeos é ainda mais rara. O presente estudo relata a ocorrência de gêmeas idênticas discordantes quanto à doença de Cushing. Uma das gêmeas, desenvolveu síndrome de Cushing aos 10 anos de idade. Sua investigação demonstrou cortisol urinário elevado, ACTH sérico elevado e testes de supressão com dexametasona compatíveis com doença de Cushing. A RMN mostrou macroadenoma hipofisário que foi operado por via transesfenoidal. O estudo imuno-histoquímico confirmou a presença de células produtoras de ACTH. A paciente entrou em remissão clínica e laboratorial desde a cirurgia. Após o desaparecimento dos estigmas do Cushing, a paciente reiniciou ganho estatural, mas permanece menor que sua irmã gêmea. Esta última permanece saudável 4 anos após o início da doença de sua irmã. Este é o terceiro caso do gênero relatado na literatura. Estes achados sugerem que fatores não genéticos estão envolvidos na gênese da doença de Cushing.Cushing's disease is rare in children and its ocurrence in identical twins is extremely rare. This paper reports on identical twins discordant for Cushing's disease. One of them first presented with a cushingoid phenotype by the age of 10. Her evaluation showed an increased urinary free-cortisol and serum ACTH. Her pattern in the dexametazone supression tests was compatible with Cushing's disease. MRI disclosed a pituitary macroadenoma which was removed by the transesphenoidal approach. Immunohistochemical studies of the tumor showed the presence of ACTH-producing cells. The patient went into clinical and laboratorial remission after surgery. She re-started to grow after the disappearance of the Cushing's phenotype but she is still shorter than her healthy sister. The latter remains disease-free 4 years after her sister's diagnosis. This represents the third such case reported in the literature. Our findings suggest that acquired

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

    Institute of Scientific and Technical Information of China (English)

    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

  15. The History of Acromegaly.

    Science.gov (United States)

    de Herder, Wouter W

    2016-01-01

    Pierre Marie coined the term 'acromegaly' in 1886 and linked it to a distinct clinical disease with a characteristic clinical picture. However, Pierre Marie was not the first physician to give a full record of the clinical picture of acromegaly; others had preceded him, like the Dutch physician Johannes Wier. After Marie, pituitary enlargement was noted in almost all patients with acromegaly. Subsequently it was discovered that pituitary hyperfunction caused by a pituitary tumour was indeed the cause of acromegaly. The cause of acromegaly could be further determined after the discovery of growth hormone (GH) and insulin-like growth factor I (IGF-I) and after demonstrating an association with GH hypersecretion and elevated circulating IGF-I. From the beginning of the 20th century, acromegaly could be treated by pituitary surgery and/or radiotherapy. After 1970, medical therapies were introduced that could control acromegaly. First, dopamine agonists were introduced, followed by somatostatin analogues and GH receptor blockers.

  16. Disease control and treatment modalities have impact on quality of life in acromegaly evaluated by Acromegaly Quality of Life (AcroQoL) Questionnaire.

    Science.gov (United States)

    Vandeva, Silvia; Yaneva, Maria; Natchev, Emil; Elenkova, Atanaska; Kalinov, Krasimir; Zacharieva, Sabina

    2015-08-01

    Various factors influence quality of life (QoL) in acromegaly. Whether disease control and treatment approach are related to QoL is still a matter of debate. The aim of the present study was to evaluate QoL in patients with acromegaly using the disease-specific Acromegaly Quality of Life Questionnaire in respect to disease activity, treatment modalities, and other factors. We studied 212 patients with acromegaly in a cross-sectional manner over a 6-year period in a single tertiary center. As a second step, seventy of the patients who were with active disease at baseline were followed up prospectively and 45 of them were in remission at re-evaluation. In regard to the cross-sectional group, active acromegaly independently predicted worse appearance scores. Prior radiotherapy and older age were independent negative predictors of all scales. Female gender negatively predicted all scales except the appearance domain. Longer duration of remission predicted worse personal relations scores in biochemically controlled patients. The use of somatostatin analog (SSA) was associated with worse personal relations scores, while higher IGF-1 index predicted worse appearance scores in patients with active acromegaly. In the prospective group, achievement of remission independently predicted improvement of the total scale. Lower corresponding baseline scores predicted improvement of the total, physical, and appearance scales, while the absence of hypopituitarism independently predicted improvement of the appearance scale. The use of SSA was associated with improvement of the total and appearance scores. In conclusion, QoL is a multifactorial issue that needs an individualized approach for detection and management.

  17. Cushing syndrome

    Science.gov (United States)

    Hypercortisolism; Cortisol excess; Glucocorticoid excess - Cushing syndrome ... The most common cause of Cushing syndrome is taking too much ... exogenous Cushing syndrome . Prednisone, dexamethasone, and ...

  18. Disease activity and lifestyle influence comorbidities and cardiovascular events in patients with acromegaly.

    Science.gov (United States)

    Sardella, Chiara; Cappellani, Daniele; Urbani, Claudio; Manetti, Luca; Marconcini, Giulia; Tomisti, Luca; Lupi, Isabella; Rossi, Giuseppe; Scattina, Ilaria; Lombardi, Martina; Di Bello, Vitantonio; Marcocci, Claudio; Martino, Enio; Bogazzi, Fausto

    2016-11-01

    The primary objective of this study is to identify the predictors of comorbidities and major adverse cardiovascular events (MACE) that can develop after diagnosis of acromegaly. The role of therapy for acromegaly in the event of such complications was also evaluated. Retrospective cohort study was conducted on 200 consecutive acromegalic patients in a tertiary referral center. The following outcomes were evaluated: diabetes, hypertension and MACE. Each patient was included in the analysis of a specific outcome, unless they were affected when acromegaly was diagnosed, and further classified as follows: (i) in remission after adenomectomy (Hx), (ii) controlled by somatostatin analogues (SSA) (SSAc) or (iii) not controlled by SSA (SSAnc). Data were evaluated using Cox regression analysis. After diagnosis of acromegaly, diabetes occurred in 40.8% of patients. The SSAnc group had a three-fold higher risk of diabetes (HR: 3.32, P = 0.006), whereas the SSAc group had a 1.4-fold higher risk of diabetes (HR: 1.43, P = 0.38) compared with the Hx group. Hypertension occurred in 35.5% of patients, after diagnosis. The determinants of hypertension were age (HR: 1.06, P = 0.01) and BMI (HR: 1.05, P = 0.01). MACE occurred in 11.8% of patients, after diagnosis. Age (HR: 1.09, P = 0.005) and smoking habit (HR: 5.95, P = 0.01) were predictors of MACE. Conversely, therapy for acromegaly did not influence hypertension or MACE. After diagnosis of acromegaly, control of the disease (irrespective of the type of treatment) and lifestyle are predictors of comorbidities and major adverse cardiovascular events. © 2016 European Society of Endocrinology.

  19. Cabergoline for Cushing's disease: a large retrospective multicenter study.

    Science.gov (United States)

    Ferriere, A; Cortet, C; Chanson, P; Delemer, B; Caron, P; Chabre, O; Reznik, Y; Bertherat, J; Rohmer, V; Briet, C; Raingeard, I; Castinetti, F; Beckers, A; Vroonen, L; Maiter, D; Cephise-Velayoudom, F L; Nunes, M L; Haissaguerre, M; Tabarin, A

    2017-03-01

    The efficacy of cabergoline in Cushing's disease (CD) is controversial. The aim of this study was to assess the efficacy and tolerability of cabergoline in a large contemporary cohort of patients with CD. We conducted a retrospective multicenter study from thirteen French and Belgian university hospitals. Sixty-two patients with CD received cabergoline monotherapy or add-on therapy. Symptom score, biological markers of hypercortisolism and adverse effects were recorded. Twenty-one (40%) of 53 patients who received cabergoline monotherapy had normal urinary free cortisol (UFC) values within 12 months (complete responders), and five of these patients developed corticotropic insufficiency. The fall in UFC was associated with significant reductions in midnight cortisol and plasma ACTH, and with clinical improvement. Compared to other patients, complete responders had similar median baseline UFC (2.0 vs 2.5xULN) and plasma prolactin concentrations but received lower doses of cabergoline (1.5 vs 3.5 mg/week, P 12 months), cabergoline was withdrawn in 28% of complete responders because of treatment escape or intolerance. Overall, sustained control of hypercortisolism was obtained in 23% of patients for 32.5 months (19-105). Nine patients on steroidogenesis inhibitors received cabergoline add-on therapy for 19 months (1-240). Hypercortisolism was controlled in 56% of these patients during the first year of treatment with cabergoline at 1.0 mg/week (0.5-3.5). About 20-25% of CD patients are good responders to cabergoline therapy allowing long-term control of hypercortisolism at relatively low dosages and with acceptable tolerability. No single parameter, including the baseline UFC and prolactin levels, predicted the response to cabergoline. © 2017 European Society of Endocrinology.

  20. Ketoconazole in Cushing's disease: is it worth a try?

    Science.gov (United States)

    Castinetti, Frederic; Guignat, Laurence; Giraud, Pauline; Muller, Marie; Kamenicky, Peter; Drui, Delphine; Caron, Philippe; Luca, Fiorina; Donadille, Bruno; Vantyghem, Marie Christine; Bihan, Helene; Delemer, Brigitte; Raverot, Gerald; Motte, Emmanuelle; Philippon, Melanie; Morange, Isabelle; Conte-Devolx, Bernard; Quinquis, Laurent; Martinie, Monique; Vezzosi, Delphine; Le Bras, Maelle; Baudry, Camille; Christin-Maitre, Sophie; Goichot, Bernard; Chanson, Philippe; Young, Jacques; Chabre, Olivier; Tabarin, Antoine; Bertherat, Jerome; Brue, Thierry

    2014-05-01

    The use of ketoconazole has been recently questioned after warnings from the European Medicine Agencies and the Food and Drug Administration due to potential hepatotoxicity. However, ketoconazole is frequently used as a drug to lower circulating cortisol levels. Several pharmacological agents have recently been approved for the treatment of Cushing's disease (CD) despite limited efficacy or significant side effects. Ketoconazole has been used worldwide for more than 30 years in CD, but in the absence of a large-scale study, its efficacy and tolerance are still under debate. We conducted a French retrospective multicenter study reviewing data from patients treated by ketoconazole as a single agent for CD, with the aim of clarifying efficacy and tolerance to better determine the benefit/risk balance. Data from 200 patients were included in this study. At the last follow-up, 49.3% of patients had normal urinary free cortisol (UFC) levels, 25.6% had at least a 50% decrease, and 25.4% had unchanged UFC levels. The median final dose of ketoconazole was 600 mg/d. Forty patients (20%) received ketoconazole as a presurgical treatment; 40% to 50% of these patients showed improvement of hypertension, hypokalemia, and diabetes, and 48.7% had normal UFC before surgery. Overall, 41 patients (20.5%) stopped the treatment due to poor tolerance. Mild (5N, superior to 5-fold normal values) increases in liver enzymes were observed in 13.5% and 2.5% of patients, respectively. No fatal hepatitis was observed. Ketoconazole is an effective drug with acceptable side effects. It should be used under close liver enzyme monitoring. Hepatotoxicity is usually mild and resolves after drug withdrawal.

  1. [Cushing's disease in childhood: apropos of a case cured after trans-sphenoidal adenomectomy].

    Science.gov (United States)

    Barrios Castellanos, R; Argente Oliver, J; Alonso Blanco, M; Yturriaga Matarranz, R

    1984-03-15

    Authors describe one case of Cushing's disease in a old eight-year boy, with growth deficiency, pubic hair and obesity. He had hypercortisolism unresponsive to dexamethasone suppression (1 mg). The more interesting fact for localization of the lesion was the more than 50% suppression with 8 mg of dexamethasone, while tomographic studies of sella turcicaland CAT were normal. A 3 mm microadenoma was removed at transsphenoidal surgery. After surgery the patient had diabetes insipida and adrenal insufficiency. One year later all endocrinologic studies were normal. This fact underlines the importance that transsphenoidal surgery can have in the treatment of Cushing's disease.

  2. Risk of Thyroid Nodular Disease and Thyroid Cancer in Patients with Acromegaly – Meta-Analysis and Systematic Review

    OpenAIRE

    Kosma Wolinski; Agata Czarnywojtek; Marek Ruchala

    2014-01-01

    INTRODUCTION: Acromegaly is a quite rare chronic disease caused by the increased secretion of growth hormone (GH) and subsequently insulin - like growth factor 1. Although cardiovascular diseases remains the most common cause of mortality among acromegalic patients, increased prevalence of malignant and benign neoplasms remains a matter of debate. The aim of this study is to evaluate the risk of thyroid nodular disease (TND) and thyroid cancer in patients with acromegaly. MATERIALS AND METHOD...

  3. ACROMEGALY: A CASE REPORT

    OpenAIRE

    -()

    2015-01-01

    Acromegaly is a rare disease caused due to hyper secretion of growth hormone. Most of the cases of acromegaly are caused by pitutary adenoma which can be microadenoma or macroadenomas. These adenomas are never malignant, but can have significant morbidity and mortality. We report a 35 year old female patient presented herewith classical presentation of acromegaly with chief complain of weight gain, excessive sweating , widening of both hands and feet and was diagnosed as a case of acromega...

  4. Arterial properties in acromegaly: relation to disease activity and associated cardiovascular risk factors.

    Science.gov (United States)

    Yaron, Marianna; Izkhakov, Elena; Sack, Jessica; Azzam, Ibrahim; Osher, Etty; Tordjman, Karen; Stern, Naftali; Greenman, Yona

    2016-06-01

    Acromegaly is associated with increased cardiovascular morbidity and mortality when inadequately treated, which may be secondary to associated comorbidities or to direct IGF-1 effects on the cardiovascular system. By using a control group carefully matched for traditional cardiovascular risk factors, we aimed to assess the direct contribution of disease activity and IGF-1 levels to arterial damage as assessed by measurements of arterial stiffness and endothelial function. Twenty-nine subjects with acromegaly (11 males, 52 ± 14 year; 15 active acromegaly) and 24 matched controls underwent evaluation of large and small artery compliance using applanation tonometry, pulse wave velocity (PWV), augmentation index (Alx), carotid ultrasonography intima-media thickness, (IMT) and flow-mediated dilatation (FMD). IGF-1 expressed as times the upper limit of the normal range (x ULN) was 2.2 ± 1.1 in patients with active disease versus 0.7 ± 0.2 in patients in remission. Irrespective of disease activity, FMD was lower in patients with acromegaly than in control subjects, (3.4 ± 2.7 % in active acromegaly, 4.4 ± 3.3 % in controlled acromegaly and 7.5 ± 3.8 % in controls; p = 0.004). There were no significant differences in PWV, Alx, and IMT between groups. A positive correlation was found between IGF-1× ULN and IMT (r = 0.4; P = 0.02). Asymmetric dimethylarginine (ADMA), a novel cardiovascular risk factor, was positively correlated to arterial stiffness (r = 0.46; p = 0.017) and negatively with small vessel compliance (r = -0.44, p = 0.02). Patients with acromegaly have significantly impaired endothelial function as assessed by FMD, but other tested vascular parameters were similar to a control group that was adequately matched for cardiovascular risk factors.

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

    Directory of Open Access Journals (Sweden)

    Priyadarshini Mishra

    2017-01-01

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

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

    Science.gov (United States)

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

    2016-02-01

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

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

    NARCIS (Netherlands)

    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

  8. Bone mineral density and turnover in patients with acromegaly in relation to sex, disease activity, and gonadal function.

    Science.gov (United States)

    Bolanowski, Marek; Daroszewski, Jacek; Medraś, Marek; Zadrozna-Sliwka, Beata

    2006-01-01

    Acromegaly is a rare disease caused by growth hormone (GH) hypersecretion. GH and insulin-like growth factor-I (IGF-I) exert anabolic activity in bones. Nevertheless, bone mineral density (BMD) loss is not uncommon in patients with acromegaly. It is assumed to be due to hypogonadism associated with the acromegaly. The aim of the study was to examine BMD at various skeletal sites and bone turnover and to assess the influence of impaired gonadal function and disease activity on BMD and turnover changes in acromegaly. A total of 62 patients were studied (40 women, 22 men). Among the women, 22 had active disease and 18 were cured; 16 women had normal gonadal function, and 24 were hypogonadal. Altogether, 12 men presented with active acromegaly, and 10 were cured; normal gonadal function was found in 10 men, and hypogonadism was diagnosed in 12 men. Controls were 30 healthy subjects. Densitometry using dual-energy X-ray absorptiometry of the lumbar spine, proximal femur, forearm, and total body was carried out. Bone turnover was studied based on serum osteocalcin, C-terminal collagen type 1 crosslinks, and bone alkaline phosphatase concentration. A disadvantageous effect of acromegaly on bone density was associated with hypogonadism in the distal radius (in women), the proximal femur (in men), and the total body (both sexes). An anabolic effect of GH during active acromegaly was present in the proximal femur only in men. We confirmed increased bone turnover in the presence of acromegaly, and these changes were similar regarding the activity of the disease and the gonadal status.

  9. Benign and malignant nodular thyroid disease in acromegaly. Is a routine thyroid ultrasound evaluation advisable?

    Directory of Open Access Journals (Sweden)

    Jordi L Reverter

    Full Text Available Data on the prevalence of benign and malignant nodular thyroid disease in patients with acromegaly is a matter of debate. In the last decade an increasing incidence of thyroid cancer has been reported. The aim of this study was to evaluate the prevalence of goiter, thyroid nodules and thyroid cancer in a large series of patients with acromegaly with a cross-sectional study with a control group. Six Spanish university hospitals participated. One hundred and twenty three patients (50% men; mean age 59±13 years; disease duration 6.7±7.2 years and 50 controls (51% males, mean age 58±15 years were studied. All participants underwent thyroid ultrasound and fine needle aspiration. Cytological analysis was performed in suspicious nodules between 0.5 and 1.0 cm and in all nodules greater than 1.0 cm. Goiter was more frequently found in patients than in controls (24.9 vs. 8.3%, respectively; p<0.001. Nodular thyroid disease as well as nodules greater than 1 cm were also more prevalent in acromegalic patients (64.6%, vs. 28.6%, p<0.05 and 53.3 vs. 28.6%, respectively; p<0.05, and all underwent fine needle aspiration. Suspicious cytology was detected in 4 patients and in none of the controls. After thyroidectomy, papillary thyroid carcinoma was confirmed in two cases (3.3% of patients with thyroid nodules, representing 1.6% of the entire group of patients with acromegaly (2.4% including a case with previously diagnosed papillary thyroid carcinoma. These data indicated that thyroid nodular disease and cancer are increased in acromegaly, thus justifying its routine ultrasound screening.

  10. The autopsy was conducted "Under most inauspicious circumstances:" John Turner, Harvey Cushing's case XXXII, and his unwitting contributions to the early understanding of acromegaly.

    Science.gov (United States)

    Pendleton, Courtney; Wand, Gary; Quinones-Hinojosa, Alfredo

    2010-12-01

    Harvey Cushing's monograph The Pituitary Body and Its Disorders describes Case XXXII, a 36-year-old man who presented with gigantism in 1910. The detailed post-mortem exam findings are prefaced with a cryptic statement, describing "inauspicious circumstances" surrounding the autopsy. Although contemporary biographies of Cushing have offered insight into these circumstances, the original surgical file for Case XXXII has not been previously reviewed. The original Johns Hopkins Hospital surgical records were reviewed, and the case of John Turner, who Cushing identified by name in his monograph The Pituitary Body and Its Disorders, was selected for further review. A review of the original surgical file revealed a typewritten note by Dr. Crowe, one of the surgeons who performed the post-mortem exam, with a handwritten addendum by Dr. Cushing. This document provides detail regarding the "inauspicious circumstances" surrounding the autopsy. Namely, the autopsy was conducted without permission of the family, during the funeral service, following a payment to the undertaker. The new information regarding the autopsy of John Turner offers insight into the previously incompletely described circumstances surrounding the autopsy. Additionally, the case illuminates the obligations and ethical quandaries that physician-scientists face.

  11. Pasiretide, a new kind of somatostatin analogs for treating Cushing disease%治疗库欣病的新生长抑素类似物帕瑞肽

    Institute of Scientific and Technical Information of China (English)

    张大明; 李乃适

    2012-01-01

    帕瑞肽是一种能与多受体结合的生长抑素类似物,与生长抑素受体sst1-3和sst5有高结合力.帕瑞肽能抑制GH,IGF-I和ACTH的分泌,提示其可能用于肢端肥大症与库欣病的治疗;而其在细胞试验中抑制神经内分泌肿瘤细胞增殖、在动物实验中显示能延长抑制激素分泌的时间以及类癌表达多个sst显示帕瑞肽在治疗类癌方面可能优于奥曲肽.目前帕瑞肽在肢端肥大症、类癌的Ⅱ期临床试验已经结束,有关库欣病方面Ⅲ期临床试验也已完成,3项试验证实其对于肢端肥大症、类癌是一种新的治疗手段,并成为治疗库欣病的新方法.%Pasireotide is a kind of somatostatin analogs which can bind with multiple receptors and especially has a high affinity with the somatostatin receptors sst,, sst2, sst3 and sst5. It inhibits release of GH, IGF-I and ACTH, suggesting a potency for the treatment of acromegaly and Cushing disease. Pasireotide inhibits proliferation of neuroendocrine tumor cells, prolongs the inhibition duration of the hormone secretion and carcinoid expressing multiple somatostatin receptors, which may have advantage in carcinoid treatment. Currently, the phrase IJ clinical trails of pasireotide in acromegaly and carcinoid have been completed, and the phrase III in Cushing disease has been accomplished too, all of which suggest that pasireotide holds the promise as a new treatment for acromegaly and carcinoid, and the first medicine for Cushing disease.

  12. Erectile Dysfunction is Common among Men with Acromegaly and is Associated with Morbidities Related to the Disease.

    Science.gov (United States)

    Lotti, Francesco; Rochira, Vincenzo; Pivonello, Rosario; Santi, Daniele; Galdiero, Mariano; Maseroli, Elisa; Balestrieri, Antonio; Faustini-Fustini, Marco; Peri, Alessandro; Sforza, Alessandra; Colao, Annamaria; Maggi, Mario; Corona, Giovanni

    2015-05-01

    The prevalence of erectile dysfunction (ED) and its correlates in men with acromegaly has never been investigated. The aim of this study was to evaluate sexual function in men with acromegaly. Multicenter-based, retrospective analysis of a nonselected series of 57 acromegalic subjects (mean age: 52.7 ± 14.2 years) was performed. Acromegalic subjects reporting ED (n = 24) were compared with matched ED patients without acromegaly or pituitary disease (controls), selected from a cohort of more than 4,000 subjects enrolled in the Florence Sexual Medicine and Andrology Unit. Patients were interviewed using Structured Interview on Erectile Dysfunction (SIEDY) structured interview, a 13-item tool for the assessment of ED-related morbidities. Several clinical and biochemical parameters were taken. Penile color Doppler ultrasound (PCDU) was performed in a subgroup of 37 acromegalic subjects. ED was reported by 42.1% of acromegalic subjects. After adjusting for age and testosterone, acromegalic subjects with ED had a higher prevalence of hypertension and more often reported an impairment of sleep-related erections and a longer smoking habit. Accordingly, acromegaly-associated ED was characterized by a higher organic component and worse PCDU parameters. No relationship between ED and testosterone levels or other acromegaly-related parameters was found. However, acromegalic subjects with severe ED reported a longer disease duration. In a case-control analysis, comparing acromegalic subjects with ED-matched controls free from acromegaly (1:5 ratio), acromegalic men had a worse ED problem and a higher organic component of ED, as derived from SIEDY score. In line with these data, acromegalic patients with ED had a higher prevalence of major adverse cardiovascular events history at enrollment and lower PCDU parameters. Subjects with complicated acromegaly are at an increased risk of developing ED, especially those with cardiovascular morbidities. Our data suggest

  13. Medical therapy in acromegaly.

    LENUS (Irish Health Repository)

    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.

  14. Progestogens and Cushing's syndrome.

    LENUS (Irish Health Repository)

    Harte, C

    2012-02-03

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

  15. Cardiovascular complications in acromegaly.

    Science.gov (United States)

    Vitale, G; Pivonello, R; Lombardi, G; Colao, A

    2004-09-01

    Cardiovascular morbidity and mortality are increased in acromegaly. In fact, GH and IGF-I excess induces a specific cardiomyopathy. The early stage of acromegaly is characterized by the hyperkinetic syndrome (high heart rate and increased systolic output). Frequently, concentric biventricular hypertrophy and diastolic dysfunction occur in acromegaly, leading to an impaired systolic function ending in heart failure if the disease is untreated or unsuccessfully untreated. Besides, abnormalities of cardiac rhythm and of valves have been also described in acromegaly. The coexistence of other complications, such as arterial hypertension and diabetes, aggravates the acromegalic cardiomyopathy. The suppression of GH/IGF-I following an efficacious therapy could decrease left ventricular mass and improve cardiac function. In conclusion, a careful evaluation of cardiac function, morphology and activity seems to be mandatory in acromegaly.

  16. Pituitary function following megavoltage therapy for Cushings' disease; long term follow up

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    Sharpe, G.F.; Kendall-Taylor, P.; Prescott, R.W.G.; Ross, W.M.; Davison, C.; Watson, M.J.; Cook, D.B. (Royal Victoria Infirmary, Newcastle upon Tyne (UK))

    1985-02-01

    Eight patients who had received megavoltage therapy for Cushings' disease 5-12 years previously have been reviewed. The long term response to this therapy was assessed with respect to efficacy of treatment in inducing continued remission and disturbance of hypothalamic-pituitary function. One patient showed clear evidence of relapse of Cushings' disease. One patient had unequivocal hypopituitarism. Basal levels of growth hormone (GH), TSH, LH, and FSH were not statistically different from controls, but provocative testing revealed significant abnormalities of response of cortisol/ACTH, GH, prolactin and LH. Six out of eight patients had absent diurnal cortisol variation and five patients had elevated serum prolactin levels. Thus, in this group of patients normal pituitary-adrenal function has not been satisfactorily restored. It is clear that significant disturbances of hypothalamic-pituitary function follow megavoltage therapy and these may progress to overt hypopituitarism.

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

    Science.gov (United States)

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

    2010-01-01

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

  18. Cushing syndrome: update on testing.

    Science.gov (United States)

    Raff, Hershel

    2015-03-01

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

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

    Energy Technology Data Exchange (ETDEWEB)

    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.

  20. ACROMEGALY: A CASE REPORT

    Directory of Open Access Journals (Sweden)

    10.5958/2319-5886.2015.00183.6

    2015-10-01

    Full Text Available Acromegaly is a rare disease caused due to hyper secretion of growth hormone. Most of the cases of acromegaly are caused by pitutary adenoma which can be microadenoma or macroadenomas. These adenomas are never malignant, but can have significant morbidity and mortality. We report a 35 year old female patient presented herewith classical presentation of acromegaly with chief complain of weight gain, excessive sweating , widening of both hands and feet and was diagnosed as a case of acromegaly due to macroadenoma of pirtutary gland, on the basis of typical clinical features and hormonal parameters also radiological findings. Patient underwent transsphenoidal surgical resection of macroadenoma and recovered completely from the disease. Early recognition and diagnosis will help to avoid the complications of disease.

  1. Acromegaly: the disease, its impact on patients, and managing the burden of long-term treatment

    Directory of Open Access Journals (Sweden)

    Adelman DT

    2013-01-01

    Full Text Available Daphne T Adelman1, Karen JP Liebert2, Lisa B Nachtigall2, Michele Lamerson3, Bert Bakker31Division of Endocrinology, Metabolism and Molecular Medicine, Feinberg School of Medicine, Northwestern University, Chicago, IL, 2Neuroendocrine Unit, Massachusetts General Hospital, Boston, MA, 3Endocrinology Medical Affairs, Ipsen Biopharmaceuticals, Inc, Basking Ridge, NJ, USAAbstract: Acromegaly is a rare disease most often caused by the prolonged secretion of excess growth hormone from a pituitary adenoma. The disease is associated with multiple significant comorbidities and increased mortality. The delay to diagnosis is often long. This may be because of low disease awareness among health care professionals, the insidious onset of differentiating features, and because patients are likely to present with complaints typical of other conditions more frequently seen in primary care. Early identification of acromegaly facilitates prompt treatment initiation and may minimize the permanent effects of excess growth hormone. The primary treatment for many patients will be pituitary surgery, although not all patients will be eligible for surgery or achieve a surgical cure. If biochemical control is not achieved following surgery, other treatment options include medical therapy and radiation therapy. Improved biochemical control may only alleviate rather than reverse the associated comorbidities. Thus, lifelong monitoring of patient health is needed, with particular attention to the management of cardiovascular risk factors. It is additionally important to consider the impact of both disease and treatment on patients' quality of life and minimize that impact where possible, but particularly for chronic therapies. For the majority of patients, chronic therapy is likely to include somatostatin analog injections. In some circumstances, it may be possible to extend the dosing interval of the analog once good biochemical control is achieved. Additional convenience

  2. Postmortem diagnosis of Fabry disease with acromegaly and a unique vasculopathy.

    Science.gov (United States)

    Takao, Masaki; Mori, Taisuke; Orikasa, Hideki; Oh, Haengphil; Suzuki, Kinuko; Koto, Atsuo; Yamazaki, Kazuto

    2007-09-01

    A 44-year-old Japanese man with elevated growth hormone levels and gradual deterioration of mental and renal function was admitted to the hospital. With his deteriorated general condition and renal failure, the patient developed pulmonary thromboembolism and died of respiratory failure. Autopsy examination was conducted, which revealed abnormal accumulation or intracytoplasmic storage of lipid-rich material in the small blood vessels, kidney, heart, and nervous system. After postmortem pathologic studies, including light-microscopic histochemistry, electron microscopy, and biochemical analysis of the stored lipid contents, a final diagnosis of Fabry disease was made. Histopathologic examination revealed a unique vasculopathy characterized by the presence of abnormal intracytoplasmic lipid inclusions and vascular remodeling. With regard to the clinical presentation of acromegaly, hyperplasia but not adenomatous transformation of the acidophils of the anterior pituitary gland with immunohistochemical detection of growth hormone within the cells was noted. In this case, the complication of acromegaly with hyperplasia of the acidophilic cells of the anterior pituitary gland and the unique vasculopathy causing significant organ failure, mainly of the kidney, heart, and central nervous systems, possibly as a result of microcirculatory failure, are considered to be not incidental findings but to be intimately involved in the pathogenesis of Farby disease.

  3. Prevalence and pathogenesis of sleep apnea and lung disease in acromegaly.

    Science.gov (United States)

    Fatti, L M; Scacchi, M; Pincelli, A I; Lavezzi, E; Cavagnini, F

    2001-09-01

    Respiratory disorders are common and important complications in acromegaly. Patients suffering from acromegaly display a 1.6-3.3 fold increase in mortality rate, which is due to respiratory disorders in 25% of cases. In these patients, mortality for lung disease is 2-3 fold higher than in the general population. Every portion of the respiratory system may be involved. Deformities of facial bones, edema and hypertrophy of the mucosae and pharyngeal and laryngeal cartilages, enlargement of the tongue and inspiratory collapse of the hypopharinx, all may contribute to respiratory alterations. Nasal polyps, "hormonal rhinitis", changes of the voice and snoring are common occurrences. Though rarely, a laryngocele may ensue. Pneumomegaly is frequently observed and, as suggested by functional studies, might be due to an increased number rather than volume of the alveoli. An obstructive respiratory syndrome caused by mucosal thickening of the upper airways and bronchi is observed in 25% of female and 70% of male patients. The sleep apnea syndrome (SAS) affects 60-70% of acromegalic patients. SAS may be of obstructive, central or mixed type. Obstructive SAS is the prevailing form in acromegaly. It is due to intermittent obstruction of upper airways with preserved activity of the respiratory center, as testified by the remarkable thoracic and abdominal respiratory efforts. The pathogenesis of the central type of SAS is more complex. Narrowing of the upper airways may induce reflex inhibition of the respiratory center. Moreover, increased GH levels and, possibly, defects in the somatostatinergic pathways, may increase the ventilatory response of the respiratory center to carbon dioxide, thereby leading to respiratory arrest. In the mixed type of SAS, the phenomena underlying the other two forms coexist. Oxygen desaturation concomitant with the apneic episodes accounts for the frequent nocturnal wakening and diurnal drowsiness. Among the clinical correlates of SAS, arterial

  4. Cushing's Syndrome

    Science.gov (United States)

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

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

    Energy Technology Data Exchange (ETDEWEB)

    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).

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

    Energy Technology Data Exchange (ETDEWEB)

    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).

  7. Associations of coefficient of variation of serum GH with previous radiotherapy, hypopituitarism and cardiac disease in patients with treated acromegaly.

    Science.gov (United States)

    Jayasena, Channa N; Izzi-Engbeaya, Chioma; Narayanaswamy, Shakunthala; Modi, Manish; Clarke, Holly; Nijher, Gurjinder M K; Meeran, Karim; Dhillo, Waljit S

    2015-06-01

    Cardiovascular complications represent the biggest cause of mortality in acromegaly. It is therefore important to optimally stratify acromegalic patients according to disease activity and complication risk. GH is secreted in a pulsatile manner from the pituitary gland, but GH pulsatility is not routinely assessed clinically. The coefficient of variation of serum GH (GHCV) during oral glucose tolerance test (OGTT) quantifies the variation of GH secretion in patients with acromegaly, but has not been reported previously. To investigate whether GHCV during OGTT is associated with clinical parameters predicted to relate with hypothalamo-pituitary dysfunction during acromegaly, such as radiotherapy treatment, pituitary deficiency and cardiac disease. GHCV was calculated during 584 OGTTs and compared with nadir serum GH and IGF-1 in 111 acromegalic patients treated at a single centre. Acromegalic patients treated with radiotherapy had a 37% lower level of GHCV when compared to the nonradiotherapy group (mean GHCV: 0·298 ± 0·015, no radiotherapy; 0·189 ± 0·007, radiotherapy; P < 0·001). Neither serum IGF-1 nor nadir GH was significantly altered in the radiotherapy group. Mean GHCV was 50% lower in the acromegalic patients with cardiac failure when compared to acromegalic patients with normal echocardiogram (0·161 ± 0·034 vs 0·297 ± 0·055; P < 0·05). Neither serum IGF-1 nor nadir GH was significantly altered during cardiac failure. Our preliminary data suggest that GHCV during OGTT may be reduced during acromegaly in patients with previous radiotherapy, pituitary deficiencies and cardiac disease. Larger studies are required to determine whether GHCV could provide help to assess the morbidity status of patients with treated acromegaly. © 2015 John Wiley & Sons Ltd.

  8. Risk of thyroid nodular disease and thyroid cancer in patients with acromegaly--meta-analysis and systematic review.

    Directory of Open Access Journals (Sweden)

    Kosma Wolinski

    Full Text Available INTRODUCTION: Acromegaly is a quite rare chronic disease caused by the increased secretion of growth hormone (GH and subsequently insulin - like growth factor 1. Although cardiovascular diseases remains the most common cause of mortality among acromegalic patients, increased prevalence of malignant and benign neoplasms remains a matter of debate. The aim of this study is to evaluate the risk of thyroid nodular disease (TND and thyroid cancer in patients with acromegaly. MATERIALS AND METHODS: PubMed, Cochrane Library, Scopus, Cinahl, Academic Search Complete, Web of Knowledge, PubMed Central, PubMed Central Canada and Clinical Key databases were searched to identify studies containing. Random-effects model was used to calculate pooled odds ratios and risk ratios of TND in acromegaly. Studies which not included control groups were systematically reviewed. RESULTS: TND was more frequent in acromegaly than in control groups (OR = 6.9, RR = 2.1. The pooled prevalence of TND was 59.2%. Also thyroid cancer (TC proved to be more common in acromegalic patients (OR = 7.5, RR = 7.2, prevalence was 4.3%. The pooled rate of malignancy (calculated per patient was equal to 8.7%. CONCLUSIONS: This study confirms that both TND and TC occur significantly more often in acromegalic patients than in general population. These results indicate that periodic thyroid ultrasound examination and careful evaluation of eventual lesions should be an important part of follow-up of patients with acromegaly.

  9. Course of pregnancies in women with Cushing's disease treated by gamma-knife.

    Science.gov (United States)

    Ferraù, Francesco; Losa, Marco; Cotta, Oana Ruxandra; Torre, Maria Luisa; Ragonese, Marta; Trimarchi, Francesco; Cannavò, Salvatore

    2012-10-01

    Data concerning pregnancy in women with Cushing's disease treated by gamma-knife (GK) are scanty. We present and discuss the course and outcome of five pregnancies in two women with Cushing's disease (CD), the first of whom was treated only by GK, and the second one treated by surgery, GK and ketoconazole. In the first patient, pregnancy was uneventful and full-term. During gestation, plasma ACTH, serum cortisol and 24-h urinary free cortisol (UFC) levels were steady, and always in the normal range for healthy non-pregnant individuals. The newborn was healthy and normal-weight. In the second woman, two pregnancies, occurring 3 years after GK and few months after ketoconazole withdrawal, were interrupted by spontaneous abortion or placental disruption despite normal cortisol levels. This patient became again pregnant 3 years later and delivered vaginally a healthy full-term infant. Seven months after the delivery, the patient became pregnant again and at the 39th week of gestation delivered vaginally a healthy male. Hypoprolactinemia and/or central hypothyroidism occurred in both cases. In women with CD treated by GK, pregnancy can occur. However, pregnancy is at risk even when ACTH and cortisol levels are normalized by treatment. After GK, evaluation of pituitary function is mandatory due to the risk of hypopituitarism.

  10. Development of ACRODAT(®), a new software medical device to assess disease activity in patients with acromegaly.

    Science.gov (United States)

    van der Lely, Aart J; Gomez, Roy; Pleil, Andreas; Badia, Xavier; Brue, Thierry; Buchfelder, Michael; Burman, Pia; Clemmons, David; Ghigo, Ezio; Jørgensen, Jens Otto Lunde; Luger, Anton; van der Lans-Bussemaker, Joli; Webb, Susan M; Strasburger, Christian J

    2017-09-08

    Despite availability of multimodal treatment options for acromegaly, achievement of long-term disease control is suboptimal in a significant number of patients. Furthermore, disease control as defined by biochemical normalization may not always show concordance with disease-related symptoms or patient's perceived quality of life. We developed and validated a tool to measure disease activity in acromegaly to support decision-making in clinical practice. An international expert panel (n = 10) convened to define the most critical indicators of disease activity. Patient scenarios were constructed based on these chosen parameters. Subsequently, a panel of 21 renowned endocrinologists at pituitary centers (Europe and Canada) categorized each scenario as stable, mild, or significant disease activity in an online validation study. From expert opinion, five parameters emerged as the best overall indicators to evaluate disease activity: insulin-like growth factor I (IGF-I) level, tumor status, presence of comorbidities (cardiovascular disease, diabetes, sleep apnea), symptoms, and health-related quality of life. In the validation study, IGF-I and tumor status became the predominant parameters selected for classification of patients with moderate or severe disease activity. If IGF-I level was ≤1.2x upper limit of normal and tumor size not significantly increased, the remaining three parameters contributed to the decision in a compensatory manner. The validation study underlined IGF-I and tumor status for routine clinical decision-making, whereas patient-oriented outcome measures received less medical attention. An Acromegaly Disease Activity Tool (ACRODAT) is in development that might assist clinicians towards a more holistic approach to patient management in acromegaly.

  11. Reversible "brain atrophy" in patients with Cushing's disease

    OpenAIRE

    Gnjidić, Živko; Sajko, Tomislav; Kudelić, Nenad; Malenica, Maša; Vizner, Branka; Vrkljan, Milan; Hat, Josip; Rumboldt, Zoran

    2008-01-01

    During the past 25 years, we came across 60 patients with corticotroph pituitary adenomas and Cushing’s disease. Neuroradiological examination showed prominent volume loss of the brain parenchyma, unexpected for the patient’s age. This »brain atrophy« appeared to regress after surgical removal of pituitary adenoma and normalization of cortisol level. Observed difference between degree of »brain atrophy« in the Cushing’s disease group and in the control group was statistically sign...

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

    DEFF Research Database (Denmark)

    Höybye, Charlotte; Ragnarsson, Oskar; Jönsson, Peter J;

    2010-01-01

    Patients in remission from Cushing's disease (CD) have many clinical features that are difficult to distinguish from those of concomitant GH deficiency (GHD). In this study, we evaluated the features of GHD in a large cohort of controlled CD patients, and assessed the effect of GH treatment....

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

    Science.gov (United States)

    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.

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

    Energy Technology Data Exchange (ETDEWEB)

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

    1980-05-01

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

  15. Known VDR polymorphisms are not associated with bone mineral density measures in pediatric Cushing disease.

    Science.gov (United States)

    Lodish, Maya B; Mastroyannis, Spyridon A; Sinaii, Ninet; Boikos, Sosipatros A; Stratakis, Constantine A

    2012-01-01

    Decreased bone mineral density (BMD) has been documented in adults with Cushing disease (CD), and allelic variants of the vitamin D receptor (VDR) gene have been associated with osteopenia. Genetic factors play an important role in bone accrual and its response to various diseases; among them, the most studied are the allelic variants of the VDR gene. There is debate as to whether described variants in the VDR gene have an effect on BMD. In the current study, we sought to analyze whether BMD differences in patients with CD were associated with the Taq1 and Apal VDR allelotypes. The data showed lack of association between BMD and these widely studied VDR polymorphisms, suggesting that the effect of endogenous hypercortisolism on bone in the context of CD does not depend on VDR genotypes.

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

    Directory of Open Access Journals (Sweden)

    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

  17. [Cyclic Cushing's Syndrome - rare or rarely recognized].

    Science.gov (United States)

    Kiałka, Marta; Doroszewska, Katarzyna; Mrozińska, Sandra; Milewicz, Tomasz; Stochmal, Ewa

    2015-01-01

    Cyclic Cushing's syndrome is a type of Cushing's disease which is characterized by alternating periods of increasing and decreasing levels of cortisol in the blood. The diagnostic criteria for cyclic Cushing's syndrome are at least three periods of hypercortisolism alternating with at least two episodes of normal levels of serum cortisol concentration. The epidemiology, signs, symptoms, pathogenesis and treatment of cyclic Cushing's syndrome have been discussed.

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

    Science.gov (United States)

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

    2017-07-01

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

  19. Volumetric MRI analysis of hippocampal subregions in Cushing's disease: a model for glucocorticoid neural modulation.

    Science.gov (United States)

    Toffanin, T; Nifosì, F; Follador, H; Passamani, A; Zonta, F; Ferri, G; Scanarini, M; Amistà, P; Pigato, G; Scaroni, C; Mantero, F; Carollo, C; Perini, G I

    2011-01-01

    Several preclinical studies have demonstrated neuronal effects of glucocorticoids on the hippocampus (HC), a limbic structure with anterior-posterior anatomical and functional segmentation. We propose a volumetric magnetic resonance imaging analysis of hippocampus head (HH), body (HB) and tail (HT) using Cushing's disease (CD) as model, to investigate whether there is a differential sensitivity to glucocorticoid neuronal damage in these segments. We found a significant difference in the HH bilaterally after 12 months from trans-sphenoidal surgical selective resection of the adrenocorticotropic hormone (ACTH)-secreting pituitary micro-adenomas. This pre-post surgery difference could contribute to better understand the pathopysiology of CD as an in vivo model for stress-related hypercortisolemic neuropsychiatric disorders.

  20. Clinical Guidelines for the Diagnosis and Treatment of Cushing's Disease in Korea

    Directory of Open Access Journals (Sweden)

    Kyu Yeon Hur

    2015-03-01

    Full Text Available Cushing's disease (CD is a rare disorder characterized by the overproduction of adrenocorticotropic hormone due to a pituitary adenoma that ultimately stimulates excessive cortisol secretion from the adrenal glands. Prior to the detection of pituitary adenomas, various clinical signs of CD such as central obesity, moon face, hirsutism, and facial plethora are usually already present. Uncontrolled hypercortisolism is associated with metabolic, cardiovascular, and psychological disorders that result in increased mortality. Hence, the early detection and treatment of CD are not only important but mandatory. Because its clinical manifestations vary from patient to patient and are common in other obesity-related conditions, the precise diagnosis of CD can be problematic. Thus, the present set of guidelines was compiled by Korean experts in this field to assist clinicians with the screening, diagnoses, and treatment of patients with CD using currently available tests and treatment modalities.

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

    Science.gov (United States)

    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.

  2. Cushing disease

    Science.gov (United States)

    ... much ACTH. ACTH stimulates production and release of cortisol, a stress hormone. Too much ACTH causes the adrenal glands to make too much cortisol. Cortisol is normally released during stressful situations. It ...

  3. Comorbidity and cardiovascular risk factors in adult GH deficiency following treatment for Cushing's disease or non-functioning pituitary adenomas during childhood

    DEFF Research Database (Denmark)

    Ragnarsson, Oskar; Höybye, Charlotte; Jönsson, Peter J;

    2012-01-01

    Cushing's disease (CD) and non-functioning pituitary adenoma (NFPA) are rare in paediatric patients. The aim of this study was to describe long-term consequences in adults with GH deficiency (GHD) treated for CD or NFPA during childhood.......Cushing's disease (CD) and non-functioning pituitary adenoma (NFPA) are rare in paediatric patients. The aim of this study was to describe long-term consequences in adults with GH deficiency (GHD) treated for CD or NFPA during childhood....

  4. Treatment update on acromegaly

    Directory of Open Access Journals (Sweden)

    Tatiana Hotimsky Millner

    2004-12-01

    Full Text Available Pegvisomant is a new drug that blocks growth hormone peripheralreceptors, allowing normalization of insulin-like growth factor-1levels and consequently of clinical symptoms, reducing morbidityand mortality associated with acromegaly in almost all patients.Its introduction made us review the literature aiming to comparethe currently available therapies for acromegaly and theirrespective advantages and disadvantages. The treatmentalgorithms for this disease were reviewed considering that thedifferent existing therapies allow more adequate individualizationto each specific case, and addressing cost, availability, efficacyand safety.

  5. Patología cardiovascular en la acromegalia Cardiovascular Disease in Acromegaly

    Directory of Open Access Journals (Sweden)

    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.

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

    OpenAIRE

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

  7. Aromatase enzyme expression in acromegaly and its possible relationship with disease prognosis.

    Science.gov (United States)

    Selek, Alev; Cetinarslan, Berrin; Gurbuz, Yesim; Tarkun, Ilhan; Canturk, Zeynep; Cabuk, Burak

    2015-05-01

    The purpose of this study was to evaluate aromatase enzyme expression in growth hormone (GH) secreting adenomas and comparison with prolactinomas, nonfunctional adenomas, and normal pituitary tissues. Also the impact of its expression on clinical and prognostic features was evaluated. 38 acromegaly, 26 prolactinoma, and 31 nonfunctional pituitary adenoma and 11 normal pituitary gland samples from autopsies were included. Aromatase and estrogen receptor-alpha (ERα) were evaluated by Immunohistochemical method; demographic, pre- and postoperative features of the patients were noted. Aromatase was expressed in varying degrees in all cases in study including controls. Aromatase expression in patients with acromegaly was significantly higher than patients with prolactinoma, nonfunctional adenoma, and controls (p = 0.04, p = 0.01 and p acromegaly, aromatase expression was negatively correlated with ER-alpha (p = 0.02, r = -0.34). Also, Ki-67 immunohistochemical results were negatively correlated with aromatase expression (p = 0.03, r = -0.27) while positively correlated with ER expression (p acromegaly. In patients with acromegaly and prolactinoma, aromatase expression was negatively correlated with Ki-67 score, and also it was higher in patients with complete postoperative remission than without remission. Therefore, aromatase expression may be a good prognostic marker predominantly in acromegaly.

  8. The value of prolactin in inferior petrosal sinus sampling with desmopressin stimulation in Cushing's disease.

    Science.gov (United States)

    Qiao, Xiaona; Ye, Hongying; Zhang, Xiaolong; Zhao, Weiwei; Zhang, Shuo; Lu, Bin; Wang, Xuanchun; Zhang, Zhaoyun; Wu, Xi; He, Min; Zhao, Xiaolong; Li, Shiqi; Zhou, Linuo; Yang, Yehong; Hu, Renming; Li, Yiming

    2015-03-01

    Prolactin may reduce false-negative results in diagnosing Cushing's disease (CD) during inferior petrosal sinus sampling (IPSS). Prolactin normalization could improve the accuracy of IPSS in predicting adenoma lateralization in CD. However, none of the previous studies had involved the use of desmopressin during IPSS. Our objective was to examine the utility of prolactin measurement during IPSS with desmopressin stimulation. We conducted a retrospective analysis of 40 patients (including 31 females) with ACTH-dependent Cushing's syndrome who underwent IPSS between 2010 and 2013. Thirty-eight CD patients were partitioned into true positive (n = 35) and false negative (n = 3). The proportion of improper IPSS venous sampling defined by corresponding IPS:P (inferior petrosal sinus to peripheral) prolactin ratio prolactin-normalized ACTH IPS:P ratio >0.8 cutoff could increase the sensitivity of IPSS to 38/38 (100 %). Among the 31 patients with histopathologically proven adenoma localization, correct prediction of adenoma lateralization was obtained in 14/31 (45 %) patients by a peak intersinus ACTH gradient of ≥1.4 in baseline and was not improved by desmopressin stimulation. Left-right intersinus gradients of unilateral prolactin-adjusted ACTH IPS:P ratios could increase the correct prediction of adenoma lateralization to 20/31 (65 %) in baseline and 24/31 (77 %) (P = 0.006) after desmopressin stimulation, respectively. Prolactin is helpful to adjust negative results of IPSS with desmopressin stimulation. It may improve the accuracy in predicting adenoma lateralization in CD as well.

  9. Exogenous Cushing syndrome

    Science.gov (United States)

    Cushing syndrome - corticosteroid induced; Corticosteroid-induced Cushing syndrome; Iatrogenic Cushing syndrome ... Cushing syndrome is a disorder that occurs when your body has a higher than normal level of the ...

  10. Acromegaly : irreversible clinical consequences

    NARCIS (Netherlands)

    Wassenaar, Monica Johanna Elisabeth

    2010-01-01

    This thesis describes the long-term consequences of growth hormone and insulin-like growth factor I excess in patients cured from acromegaly for a mean duration of 17 years. Regarding the considerable prevalence of diverse morbidity in these patients, during the active phase of the disease but even

  11. Acromegaly : irreversible clinical consequences

    NARCIS (Netherlands)

    Wassenaar, Monica Johanna Elisabeth

    2010-01-01

    This thesis describes the long-term consequences of growth hormone and insulin-like growth factor I excess in patients cured from acromegaly for a mean duration of 17 years. Regarding the considerable prevalence of diverse morbidity in these patients, during the active phase of the disease but even

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

    Science.gov (United States)

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

    2008-07-01

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

  13. Brain glucose metabolism is associated with hormone level in Cushing's disease: A voxel-based study using FDG-PET

    OpenAIRE

    Shuai Liu; Yinyan Wang; Kaibin Xu; Fan Ping; Renzhi Wang; Fang Li; Xin Cheng

    2016-01-01

    Chronic exposure to elevated levels of glucocorticoids can exert a neurotoxic effect in patients, possibly manifesting as molecular imaging alterations in patients. The aim of this study was to investigate the potential association between brain metabolism and elevated hormone level using 18F-fluorodeoxyglucose positron emission tomography. We retrospectively enrolled 92 consecutive patients with confirmed diagnosis of Cushing's disease. A voxel-based analysis was performed to investigate the...

  14. The molecular pathogenesis of Cushing's disease%库欣病的分子发病机制

    Institute of Scientific and Technical Information of China (English)

    魏薇; 王卫庆

    2014-01-01

    Cushing's disease is a condition of an excess of the steroid hormone cortisol in the blood caused by a pituitary corticotropic adenoma secreting adrenocorticotropic hormone.Cushing's disease in familial endocrine syndromes with pituitary adenomas is related to the gene mutation of MEN-1 and GNAS1.And the abnormal expression of the PTTG and p27 genes,hormone related receptors and ligands are involved in the pathogenesis of sporadic Cushing's disease,which cause the proliferation of the corticotroph cells and excessive ACTH secretion.Further understanding of the molecular pathogenesis of Cushing's disease is helpful in early diagnosis and targeted therapy.%库欣病是垂体促肾上腺皮质激素(ACTH)腺瘤分泌过多ACTH,刺激肾上腺过度合成和分泌糖皮质激素所致.家族性内分泌综合征伴垂体ACTH腺瘤与多发性内分泌腺瘤病(MEN)-1及GNAS1基因突变相关.散发性库欣病的发病机制涉及PTTG、p27基因和激素相关受体、配体的异常表达,造成垂体ACTH细胞的过度增殖和激素分泌.进一步了解库欣病的分子发病机制对早期诊断和靶向治疗有重要意义.

  15. Gamma Knife Radiosurgery for Acromegaly

    Directory of Open Access Journals (Sweden)

    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.

  16. Cutaneous Findings in Patients with Acromegaly

    OpenAIRE

    Akoglu, Gulsen; Metin, Ahmet; Emre, Selma; Ersoy, Reyhan; Cakir, Bekir

    2014-01-01

    Background: Acromegaly is a systemic syndrome caused by overproduction of growth hormone. The syndrome affects cutaneous, endocrine, cardiovascular, skeletal, and respiratory systems. Cutaneous manifestations of acromegaly are various, usually being the first presenting findings of the disease. Methods: Dermatological examinations of 49 patients of acromegaly who were followed-up at a tertiary referral hospital.Results: The study included 27 (55.1%) female and 22 (44.9%) male patients. The on...

  17. Reversible posterior encephalopathy syndrome associated with micronodular adrenocortical disease and Cushing syndrome.

    Science.gov (United States)

    Lodish, Maya; Patronas, Nicholas J; Stratakis, Constantine A

    2010-01-01

    We report a 6-year-old girl with ACTH-independent Cushing syndrome secondary to bilateral adrenal hyperplasia; she presented with hypertension and seizures, and magnetic resonance imaging shows changes consistent with posterior reversible encephalopathy syndrome.

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

    Directory of Open Access Journals (Sweden)

    M.O. Ryznychuk

    2016-04-01

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

  19. OSTEOPOROSIS IN СHILDREN WITH ITSENKO — CUSHING DISEASE: ETIOLOGY, PATHOGENESIS, DIAGNOSIS

    OpenAIRE

    Ryznychuk Mariana Oleksandrivna; KHLUNOVSKA L.YU.; KRETSU T.M.; DMITRUK V.P.; KOSTIV M.I.

    2016-01-01

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

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

    Science.gov (United States)

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

    2015-01-01

    Context: Cushing disease, due to pituitary corticotroph tumor ACTH hypersecretion, drives excess adrenal cortisol production with adverse morbidity and mortality. Loss of glucocorticoid negative feedback on the hypothalamic-pituitary-adrenal axis leads to autonomous transcription of the corticotroph precursor hormone proopiomelanocortin (POMC), consequent ACTH overproduction, and adrenal hypercortisolism. We previously reported that R-roscovitine (CYC202, seliciclib), a 2,6,9-trisubstituted purine analog, suppresses cyclin-dependent-kinase 2/cyclin E and inhibits ACTH in mice and zebrafish. We hypothesized that intrapituitary cyclin E signaling regulates corticotroph tumor POMC transcription independently of cell cycle progression. The aim was to investigate whether R-roscovitine inhibits human ACTH in corticotroph tumors by targeting the cyclin-dependent kinase 2/cyclin E signaling pathway. Methods: Primary cell cultures of surgically resected human corticotroph tumors were treated with or without R-roscovitine, ACTH measured by RIA and quantitative PCR, and/or Western blot analysis performed to investigate ACTH and lineage-specific transcription factors. Cyclin E and E2F transcription factor 1 (E2F1) small interfering RNA (siRNA) transfection was performed in murine corticotroph tumor AtT20 cells to elucidate mechanisms for drug action. POMC gene promoter activity in response to R-roscovitine treatment was analyzed using luciferase reporter and chromatin immunoprecipitation assays. Results: R-roscovitine inhibits human corticotroph tumor POMC and Tpit/Tbx19 transcription with decreased ACTH expression. Cyclin E and E2F1 exhibit reciprocal positive regulation in corticotroph tumors. R-roscovitine disrupts E2F1 binding to the POMC gene promoter and suppresses Tpit/Tbx19 and other lineage-specific POMC transcription cofactors via E2F1-dependent and -independent pathways. Conclusion: R-roscovitine inhibits human pituitary corticotroph tumor ACTH by targeting the

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

    Science.gov (United States)

    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

  2. Pregnancy and acromegaly.

    Science.gov (United States)

    Muhammad, Ammar; Neggers, Sebastian J; van der Lely, Aart J

    2017-02-01

    Acromegaly is a rare disorder in which, due to the high incidence of secondary hypogonadism, pregnancies are relatively rare. However, some women with acromegaly do get pregnant, which brings along questions about medication, complications and follow-up. This review tries to address these issues and provide the reader with practical information. This review summarizes published data. Acromegaly is a disorder that is characterized by changes in growth hormone (GH), insulin-like growth factor-1 (IGF-1) and insulin concentrations and actions. All these hormones are important in pregnancy as well. In principle, the fetal-placental collaboration between mother and child more-or-less takes over the control over GH and IGF-1, not only in normal physiology but also to a certain extend in acromegaly. When medication for the high GH levels or actions is continued during pregnancy, both dopamine agonists, somatostatin analogs and GH receptor antagonists have been used and the available data suggest that there are no adverse consequences on mother or fetus to date. However, it is strongly advised to stop any medical intervention during pregnancy until more data are available on the safety of these compounds. Also, medical treatment is not needed as tumor size and disease activity are not reported to escape.

  3. Impact of patient's age and disease duration on cardiac performance in acromegaly: a radionuclide angiography study.

    Science.gov (United States)

    Colao, A; Cuocolo, A; Marzullo, P; Nicolai, E; Ferone, D; Della Morte, A M; Petretta, M; Salvatore, M; Lombardi, G

    1999-05-01

    The aim of this study was to evaluate the impact of age and disease duration on cardiac performance in acromegaly. To address these issues, the left ventricular function at rest and during physical exercise was assessed by equilibrium radionuclide angiography in 40 rigorously selected patients with active acromegaly but without evidence of other complications able to affect heart function and in 32 healthy controls. Patients and controls were divided in two groups, on the basis of age below and above 40 yr. Circulating GH and insulin-like growth factor-I levels were significantly increased in patients, compared with controls, but were similar in the two groups of patients. At peak exercise, the systolic blood pressure was significantly higher in elderly patients (P 50%) in all but 2 patients and in all controls. The left ventricular ejection fraction at peak exercise was significantly decreased in elderly, compared with young, patients (P < 0.01) and in age-matched controls (P < 0.001). A normal response of the left ventricular ejection fraction to exercise was found in 12 of 40 patients (30%) and in 28 of 32 controls (87.5%) (chi2, 5.764; P < 0.01). Exercise-induced changes in left ventricular ejection fraction were significantly decreased in young (+5.2 +/- 4.4% vs. +21.3 +/- 3.4%, P < 0.005) and elderly patients (-10.2 +/- 2.8% vs. +13.7 +/- 2.7%, P < 0.0001), as compared with age-matched controls. The peak rate of left ventricular filling was significantly higher in young, than in elderly, patients whether peak filling rate was normalized to end-diastolic volume (P < 0.001), or stroke volume (P < 0.0001), or expressed as the ratio of peak filling rate to peak ejection rate (P < 0.001). The peak rate of left ventricular filling was significantly decreased in elderly patients, compared with young patients and age-matched controls, whether peak filling rate was normalized to end-diastolic volume (P < 0.01), or stroke volume (P < 0.005), or expressed as the ratio

  4. Characteristics of Acromegaly in Korea with a Literature Review

    OpenAIRE

    Hong, Jae Won; Ku, Cheol Ryong; Kim, Sun Ho; Lee, Eun Jig

    2013-01-01

    Acromegaly is a slowly progressive disease caused by excessive growth hormone (GH), which is related to a GH secreting pituitary tumor in most cases. Herein, we describe the epidemiology, clinical characteristics, and treatment of acromegaly in Korea with a literature review. The average annual incidence of acromegaly in Korea was 3.9 cases per million people, which was within the range of previous Western studies. The primary treatment for acromegaly was also transsphenoidal adenomectomy, wh...

  5. Results of repeated transsphenoidal surgery in Cushing's disease. Long-term follow-up.

    Science.gov (United States)

    Valderrábano, Pablo; Aller, Javier; García-Valdecasas, Leopoldo; García-Uría, José; Martín, Laura; Palacios, Nuria; Estrada, Javier

    2014-04-01

    Transsphenoidal surgery (TSS) is the treatment of choice for Cushing's disease (CD). However, the best treatment option when hypercortisolism persists or recurs remains unknown. The aim of this study was to analyze the short and long-term outcome of repeat TSS in this situation and to search for response predictors. Data from 26 patients with persistent (n=11) or recurrent (n=15) hypercortisolism who underwent repeat surgery by a single neurosurgeon between 1982 and 2009 were retrospectively analyzed. Remission was defined as normalization of urinary free cortisol (UFC) levels, and recurrence as presence of elevated UFC levels after having achieved remission. The following potential outcome predictors were analyzed: adrenal status (persistence or recurrence) after initial TSS, tumor identification in imaging tests, degree of hypercortisolism before repeat TSS, same/different surgeon in both TSS, and time to repeat surgery. Immediate postoperative remission was achieved in 12 patients (46.2%). Five of the 10 patients with available follow-up data relapsed after surgery (median time to recurrence, 13 months). New hormone deficiencies were seen in seven patients (37%), and two patients had cerebrospinal fluid leakage. No other major complications occurred. None of the preoperative factors analyzed was predictive of surgical outcome. When compared to initial surgery, repeat TSS for CD is associated to a lower remission rate and a higher risk of recurrence and complications. Further studies are needed to define outcome predictors. Copyright © 2013 SEEN. Published by Elsevier Espana. All rights reserved.

  6. Kidney Stones as an Under-Recognized Clinical Sign in Pediatric Cushing Disease

    Science.gov (United States)

    Rahman, Sara H.; Papadakis, Georgios Z.; Keil, Margaret F.; Faucz, Fabio R.; Lodish, Maya B.; Stratakis, Constantine A.

    2015-01-01

    Objective To investigate the prevalence of kidney stones in a population of children with Cushing disease (CD) and to compare this prevalence with that of healthy children. Study design Clinical and biochemical data from 139 pediatric patients with CD (68 female, 71 male) were retrospectively analyzed. Computed tomography (CT) scans were reviewed for kidney stones. Results Of 139 patients, 27 children with CD (19.4%) had either radiographic evidence and/or a history of kidney stones. Those with kidney stones had higher urine free cortisol (p-value = 0.008) and a transsphenoidal surgery at an older age (p-value = 0.007). Average urinary calcium creatinine ratio was elevated in patients with CD (0.22 ± 0.11). The prevalence of kidney stones in children with CD was higher than in normal children (19.42% vs 1.0%, p-value <0.001). Conclusion Our results illustrate that kidney stones are an under-estimated complication of pediatric CD, especially when compared with the prevalence of nephrolithiasis in the general pediatric population. Long term consequences for kidney function are not known and need to be studied. PMID:26703870

  7. Increased prevalence of psychopathology and maladaptive personality traits after long-term cure of Cushing's disease.

    Science.gov (United States)

    Tiemensma, Jitske; Biermasz, Nienke R; Middelkoop, Huub A M; van der Mast, Roos C; Romijn, Johannes A; Pereira, Alberto M

    2010-10-01

    Psychopathology and maladaptive personality traits are often observed during the active phase of Cushing's disease (CD). We hypothesized that patients with long-term cure of CD show persistent psychopathology and maladaptive personality traits. Four questionnaires on frequently occurring psychopathology in somatic illnesses were used, including the Apathy Scale, Irritability Scale, Hospital Anxiety and Depression Scale, and Mood and Anxiety Symptoms Questionnaire short-form. Personality was assessed using the Dimensional Assessment of Personality Pathology short-form (DAPPs). We included 51 patients cured of CD (16% men, 53 ± 13 yr) and 51 matched controls. In addition, we included 55 patients treated for nonfunctioning pituitary macroadenomas (55% men, 62 ± 10 yr), and 55 matched controls. Mean duration of remission was 11 yr (range 1-32 yr). Compared with matched controls, patients cured from CD scored significantly worse on virtually all questionnaires. Compared with nonfunctioning pituitary macroadenoma patients, patients treated for CD scored worse on apathy (P Personality Pathology short-form (P maladaptive personality traits. These observations suggest irreversible effects of previous glucocorticoid excess on the central nervous system rather than an effect of pituitary tumors and/or their treatment in general. This may also be of relevance for patients treated with high doses of exogenous glucocorticoids.

  8. [Primary pigmented nodular adrenocortical disease as cause of Cushing's syndrome associated with Carney complex].

    Science.gov (United States)

    Dumić, Miroslav; Janjanin, Nevena; Uroić, Anita Spehar; Ille, Jasenka; Skegro, Mate; Kusec, Vesna; Marjanac, Igor; Matić, Toni; Jelasić, Drazen

    2006-01-01

    We report a 11-year-old girl and two 14-year-old boys with Cushing's syndrome due to primary pigmented nodular adrenocortical disease (PPNAD). In these patients, hypercortisolism is a consequence of autonomous cortisol secretion from adrenal glands and is ACTH-independent. Besides PPNAD, the girl had lentigines, spotty pigmentation on her bucal mucosa and lips and she also had schwannoma. One of the reported boys had prolactinoma. Considering this, those two patients fulfill the criteria for Carney complex which is a type of multiple endocrine neoplasia syndromes inherited in an autosomal dominant trait. The other boy had PPNAD but no other obvious signs of Carney complex were noticed. Family study didn't reveal any clinical or laboratory signs of Carney complex in our patients' first relatives. All of our patients underwent bilateral adrenalectomy (in one of the boys laparoscopic surgery was performed). Glucocorticoid and mineralocorticoid substitution has been started. Adrenal glands were macroscopically normal but pathohistological analysis confirmed the diagnosis of PPNAD.

  9. Spontaneous remission of acromegaly and Cushing’s disease following pituitary apoplexy: Two case reports

    NARCIS (Netherlands)

    Roerink, S.H.P.P.; Lindert, E.J. van; Ven, A.C. van de

    2015-01-01

    In this double case report, we present two special cases of pituitary apoplexy. First, we describe a patient with growth hormone deficiency despite clinical suspicion of acromegaly. Imaging showed evidence of a recent pituitary apoplexy, which might have caused spontaneous remission of the acromegal

  10. Sleep-disordered breathing in acromegaly

    Directory of Open Access Journals (Sweden)

    L K Dzeranova

    2013-03-01

    Full Text Available Sleep-disordered breathing is higly prevalent in acromegaly, disturbing patients quality of life and increasing the risk of acute cardiovascular compications. Presented clinical case discusses key considerations for timely diagnosis of sleep apnea syndrome and treatment planning. The case of 41 y.o. woman with newly diagnosed acromegaly and concomitant sleep apnea is typical for this disease.

  11. Urinary growth hormone excretion in acromegaly

    DEFF Research Database (Denmark)

    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...

  12. [Drug therapy for acromegaly].

    Science.gov (United States)

    Góth, Miklós

    2013-09-29

    Prolonged overproduction of growth hormone, like insulin-like growth factor-1 hypersecretion leads to acromegaly in adults. This is associated with several co-morbidities and increased mortality. Despite typical clinical features and modern diagnostic tools, it often takes years to diagnose from the onset of the disease. The aims of the treatment are to reduce or control tumour growth, inhibit growth hormone hypersecretion, normalize insulin-like growth factor-1 levels, treat co-morbidities and, therefore, reduce mortality. There are three approaches for therapy: surgery, medical management (dopamine agonists, somatostatin analogues and growth hormone receptor antagonist), and radiotherapy. Efficient therapy of the disease is based on the appropriate multidisciplinary team management. The review provides a summary of medical treatment for acromegaly.

  13. Assessment of the outcomes of the treatment of Cushing's disease in the hospitals of Castilla-La Mancha.

    Science.gov (United States)

    Huguet, Isabel; Aguirre, Miguel; Vicente, Almudena; Alramadan, Mubarak; Quiroga, Iván; Silva, Julia; Lamas, Cristina

    2015-05-01

    Treatment of Cushing's disease poses interesting dilemmas in clinical practice. The aim of our study was to analyze the outcomes of the different treatments, the control and recurrence rates, and the complications derived from them. Data were collected from the clinical records of 22 patients over 18 years of age (86.4% women). They had been diagnosed with Cushing's disease between 2000 and 2012, and were monitored at Complejo Hospitalario Universitario-Albacete, Hospital Virgen de la Salud-Toledo Hospital General Universitario de Ciudad Real, Hospital Virgen de la Luz-Cuenca, Hospital Nuestra Señora del Prado-Talavera de la Reina, and Complejo Hospitalario la Mancha Centro-Alcázar de San Juan. Surgery was the treatment of choice in all patients. Biochemical cure was achieved in 72.2% of patients. Nine patients developed in the early postoperative period diabetes insipidus, which became in 2 patients only. Surprisingly, 3 patients with normal postoperative neurohypophyseal function later developed permanent diabetes insipidus. New hormone deficiencies occurred in 7 patients. Seventeen patients received ketoconazole before surgery (5 of them after surgery also), and 70% of them achieved normal urinary free cortisol levels. Three patients also received radiotherapy, and all of them were cured after a median follow-up of 85.5 months; they developed no tumors or other complications. Our study reports the outcomes of management of Cushing's disease in non-reference centers for this disease, possibly giving a realistic picture of standard clinical practice for the condition in Spain. Copyright © 2014 SEEN. Published by Elsevier España, S.L.U. All rights reserved.

  14. [Acromegaly: current view].

    Science.gov (United States)

    Kršek, Michal

    2015-10-01

    Acromegaly is a rare disorder caused by autonomous oversecretion of growth hormone mostly by pituitary adenoma. Untreated acromegaly leads to significantly increased morbidity and mortality and impaired quality of life. Early diagnosis and treatment is therefore essential for improvement of patients prognosis and management of acromegaly should be concentrated in specialized centres. Present article summarizes current view on diagnosis and treatment of acromegaly.

  15. Cabergoline in acromegaly.

    Science.gov (United States)

    Kuhn, Emmanuelle; Chanson, Philippe

    2017-02-01

    Acromegaly, a rare disease due to growth hormone (GH) hypersecretion by a pituitary adenoma, is associated with severe comorbidity and premature death if not adequately treated. The usual first-line treatment is surgery. Various drugs, including somatostatin receptor ligands, dopamine agonists and GH receptor antagonists, are now available for use if surgery fails to suppress GH/IGF-I hypersecretion. Cabergoline, now the preferred dopamine agonist for treating hyperprolactinemia, is also used off-label for treating acromegaly. Cabergoline monotherapy is reported to normalize IGF-I levels in more than one-third of patients with acromegaly. When a somatostatin receptor ligand proves ineffective, cabergoline add-on therapy normalizes the IGF-I level in 40-50% of patients. Finally, when combined with the GH receptor antagonist pegvisomant in patients with mild uncontrolled disease, cabergoline helps to achieve normal IGF-I levels while avoiding the need for high-dose pegvisomant. Cabergoline is also inexpensive and well tolerated; in particular, it does not appear to promote heart valve disease.

  16. Accuracy of Late-Night Salivary Cortisol in Evaluating Postoperative Remission and Recurrence in Cushing's Disease.

    Science.gov (United States)

    Amlashi, Fatemeh G; Swearingen, Brooke; Faje, Alexander T; Nachtigall, Lisa B; Miller, Karen K; Klibanski, Anne; Biller, Beverly M K; Tritos, Nicholas A

    2015-10-01

    Late-night salivary cortisol (LNSC) is well-validated in the diagnosis of Cushing's disease (CD). The accuracy of LNSC during follow-up of patients undergoing transsphenoidal surgery (TSS) has not been fully characterized. We examined the accuracy of LNSC in establishing remission and identifying recurrence in postoperative patients with CD. This is a retrospective study. Records of patients with CD who underwent TSS by a single neurosurgeon in our tertiary center (2005-2014) were analyzed (N = 224). Patients were selected for further investigation (n = 165) if there was at least one available LNSC test obtained after TSS (either within 3 months or during long-term follow-up). Extracted data included demographic and clinical characteristics, magnetic resonance imaging and laboratory data (morning serum cortisol, 24-hour urine free cortisol [UFC], LNSC) . Remission was defined as nadir morning serum cortisol less than 5 mcg/dl and nadir 24-hour UFC less than 23 mcg. Recurrence was considered definite if confirmed surgically or prompted radiotherapy. Surgical remission occurred in 89% of 89 patients with available LNSC data. LNSC, obtained within 3 months of TSS, established remission with 94% sensitivity and 80% specificity at a cutpoint of 1.9 nmol/l (area under the curve [AUC] = 0.90). At a median follow-up of 53.5 months, LNSC established recurrence (75% sensitivity and 95% specificity) at a cutpoint of 7.4 nmol/l (AUC = 0.87), and 24-hour UFC established recurrence (68% sensitivity and 100% specificity) at a cutpoint of 1.6-fold above normal (AUC = 0.82). LNSC may accurately establish remission after TSS and identify recurrence more accurately than 24-hour UFC during long-term follow-up.

  17. Acromegaly: clinical features at diagnosis.

    Science.gov (United States)

    Vilar, Lucio; Vilar, Clarice Freitas; Lyra, Ruy; Lyra, Raissa; Naves, Luciana A

    2017-02-01

    Acromegaly is a rare and underdiagnosed disorder caused, in more than 95% of cases, by a growth hormone (GH)-secreting pituitary adenoma. The GH hypersecretion leads to overproduction of insulin-like growth factor 1 (IGF-1) which results in a multisystem disease characterized by somatic overgrowth, multiple comorbidities, physical disfigurement, and increased mortality. This article aims to review the clinical features of acromegaly at diagnosis. Acromegaly affects both males and females equally and the average age at diagnosis ranges from 40 to 50 years (up to 5% of cases acromegaly is often diagnosed five to more than ten years after its onset. The typical coarsening of facial features include furrowing of fronthead, pronounced brow protrusion, enlargement of the nose and the ears, thickening of the lips, skin wrinkles and nasolabial folds, as well as mandibular prognathism that leads to dental malocclusion and increased interdental spacing. Excessive growth of hands and feet (predominantly due to soft tissue swelling) is present in the vast majority of acromegalic patients. Gigantism accounts for up to 5% of cases and occurs when the excess of GH becomes manifest in the young, before the epiphyseal fusion. The disease also has rheumatologic, cardiovascular, respiratory, neoplastic, neurological, and metabolic manifestations which negatively impact its prognosis and patients quality of life. Less than 15% of acromegalic patients actively seek medical attention for change in appearance or enlargement of the extremities. The presentation of acromegaly is more often related to its systemic comorbidities or to local tumor effects.

  18. Clinical Manifestations and Diagnosis of Acromegaly

    Directory of Open Access Journals (Sweden)

    Gloria Lugo

    2012-01-01

    Full Text Available Acromegaly and gigantism are due to excess GH production, usually as a result of a pituitary adenoma. The incidence of acromegaly is 5 cases per million per year and the prevalence is 60 cases per million. Clinical manifestations in each patient depend on the levels of GH and IGF-I, age, tumor size, and the delay in diagnosis. Manifestations of acromegaly are varied and include acral and soft tissue overgrowth, joint pain, diabetes mellitus, hypertension, and heart and respiratory failure. Acromegaly is a disabling disease that is associated with increased morbidity and reduced life expectancy. The diagnosis is based primarily on clinical features and confirmed by measuring GH levels after oral glucose loading and the estimation of IGF-I. It has been suggested that the rate of mortality in patients with acromegaly is correlated with the degree of control of GH. Adequately treated, the relative mortality risk can be markedly reduced towards normal.

  19. Clinical manifestations and diagnosis of acromegaly.

    Science.gov (United States)

    Lugo, Gloria; Pena, Lara; Cordido, Fernando

    2012-01-01

    Acromegaly and gigantism are due to excess GH production, usually as a result of a pituitary adenoma. The incidence of acromegaly is 5 cases per million per year and the prevalence is 60 cases per million. Clinical manifestations in each patient depend on the levels of GH and IGF-I, age, tumor size, and the delay in diagnosis. Manifestations of acromegaly are varied and include acral and soft tissue overgrowth, joint pain, diabetes mellitus, hypertension, and heart and respiratory failure. Acromegaly is a disabling disease that is associated with increased morbidity and reduced life expectancy. The diagnosis is based primarily on clinical features and confirmed by measuring GH levels after oral glucose loading and the estimation of IGF-I. It has been suggested that the rate of mortality in patients with acromegaly is correlated with the degree of control of GH. Adequately treated, the relative mortality risk can be markedly reduced towards normal.

  20. Clinical Manifestations and Diagnosis of Acromegaly

    Science.gov (United States)

    Lugo, Gloria; Pena, Lara; Cordido, Fernando

    2012-01-01

    Acromegaly and gigantism are due to excess GH production, usually as a result of a pituitary adenoma. The incidence of acromegaly is 5 cases per million per year and the prevalence is 60 cases per million. Clinical manifestations in each patient depend on the levels of GH and IGF-I, age, tumor size, and the delay in diagnosis. Manifestations of acromegaly are varied and include acral and soft tissue overgrowth, joint pain, diabetes mellitus, hypertension, and heart and respiratory failure. Acromegaly is a disabling disease that is associated with increased morbidity and reduced life expectancy. The diagnosis is based primarily on clinical features and confirmed by measuring GH levels after oral glucose loading and the estimation of IGF-I. It has been suggested that the rate of mortality in patients with acromegaly is correlated with the degree of control of GH. Adequately treated, the relative mortality risk can be markedly reduced towards normal. PMID:22518126

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

    Energy Technology Data Exchange (ETDEWEB)

    Rotte, Alexandra A.J. de; Groenewegen, Amy; Rutgers, Dik R.; Witkamp, Theo; Luijten, Peter R.; Hendrikse, Jeroen [University Medical Center Utrecht, Department of Radiology, Utrecht (Netherlands); Zelissen, Pierre M.J. [University Medical Center Utrecht, Department of Internal Medicine (Section of Endocrinology), Utrecht (Netherlands); Meijer, F.J.A. [Radboud University Medical Center, Department of Radiology and Nuclear Medicine, Nijmegen (Netherlands); Lindert, Erik J. van [Radboud University Medical Center, Department of Neurosurgery, Nijmegen (Netherlands); Hermus, Ad [Radboud University Medical Center, Department of Internal Medicine (Section of Endocrinology), Nijmegen (Netherlands)

    2016-01-15

    To evaluate the detection of pituitary lesions at 7.0 T compared to 1.5 T MRI in 16 patients with clinically and biochemically proven Cushing's disease. In seven patients, no lesion was detected on the initial 1.5 T MRI, and in nine patients it was uncertain whether there was a lesion. Firstly, two readers assessed both 1.5 T and 7.0 T MRI examinations unpaired in a random order for the presence of lesions. Consensus reading with a third neuroradiologist was used to define final lesions in all MRIs. Secondly, surgical outcome was evaluated. A comparison was made between the lesions visualized with MRI and the lesions found during surgery in 9/16 patients. The interobserver agreement for lesion detection was good at 1.5 T MRI (κ = 0.69) and 7.0 T MRI (κ = 0.62). In five patients, both the 1.5 T and 7.0 T MRI enabled visualization of a lesion on the correct side of the pituitary gland. In three patients, 7.0 T MRI detected a lesion on the correct side of the pituitary gland, while no lesion was visible at 1.5 T MRI. The interobserver agreement of image assessment for 7.0 T MRI in patients with Cushing's disease was good, and lesions were detected more accurately with 7.0 T MRI. (orig.)

  2. Long-term results of stereotactic radiosurgery to the pituitary gland in Cushing's disease

    Energy Technology Data Exchange (ETDEWEB)

    Degerblad, M.; Raehn, T.; Bergstrand, G.; Thoren, M.

    1986-01-01

    Gamma radiation from /sup 60/Co delivered with stereotactic technique was given to the pituitary gland in 35 patients, aged 18-65 years, with Cushing's disease. The doses were 70-100 Gy in each single irradiation. The size of the sella turcica was normal in the majority of the patients. The observation time was 3-9 years in 29 patiens. Out of them, 14 (48%) obtained clinical remission and normal urinary cortisol after one irradiation. Eight achieved remission after two to four irradiations. In total, 22 out of 29 patients (76%) obtained remission. In 12 of them remission was obtained in 1 year and in another 10 within 3 years. No recurrences were observed. Improvement was seen in 2 patients after one and three irradiations. Bilateral adrenalectomy was performed in 5 patients owing to unsatisfactory effect of irradiation. Pituitary insufficiency with gonadotropin, thyrotropin or cortocotropin failure was demonstrated in 12 of 22 patients in remission. This occurred 4 months to 7 years after the first irradiation. Another 6 patients were followed less than 3 years after the first irradiation. Two obtained remission after the first treatment, whereas the other 4 improved. Stereotactic pituitary irradiation is suggested as a non-invasive therapeutic alternative in Cushing's disease for example in patients with considerable surgical risk or as a supplement to pituitary microsurgery.

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

    Science.gov (United States)

    Giugni, Aldo Schenone; Mani, Shylaja; Kannan, Subramanian; Hatipoglu, Betul

    2013-01-01

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

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

    Directory of Open Access Journals (Sweden)

    Aldo Schenone Giugni

    2013-01-01

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

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

    OpenAIRE

    Aldo Schenone Giugni; Shylaja Mani; Subramanian Kannan; Betul Hatipoglu

    2013-01-01

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

  6. Reversible posterior encephalopathy syndrome associated with micronodular adrenocortical disease and Cushing syndrome

    OpenAIRE

    Lodish, Maya; Patronas, Nicholas J.; Stratakis, Constantine A.

    2009-01-01

    We report the case of a 6-year-old female with ACTH-independent Cushing syndrome secondary to bilateral adrenal nodular hyperplasia, who presented with hypertension and seizures, and was found have MRI changes consistent with posterior reversible encephalopathy syndrome (PRES). The patient received anti-hypertensive medication and a bilateral adrenalectomy was performed. One month later, resolution of her brain MRI changes were seen. This is the first case described in the literature of a pat...

  7. Cushing's disease and hypertension: In vivo and in vitro study of the role of the renin-Angiotensin-Aldosterone system and effects of medical therapy

    NARCIS (Netherlands)

    R. van der Pas (Rob); J.H.M. Esch, van (Joep); C. de Bruin (Christiaan); A.H.J. Danser (Jan); A.M. Pereira (Alberto); P.M. Zelissen (Pierre M.); R.T. Netea-Maier (Romana ); D. Sprij-Mooij (Diana); I.M. van den Berg-Garrelds (I.); R.H.N. van Schaik (Ron); S.W.J. Lamberts (Steven); A.H. van den Meiracker (Anton); L.J. Hofland (Leo); R.A. Feelders (Richard)

    2014-01-01

    textabstractAbstract Objective/methods: Cushing's disease (CD) is often accompanied by hypertension. CD can be treated surgically and, given the expression of somatostatin subtype 5 and dopamine 2 receptors by corticotroph pituitary adenomas, pharmacologically. Indeed, we recently observed that step

  8. Cushing's disease and hypertension: in vivo and in vitro study of the role of the renin-angiotensin-aldosterone system and effects of medical therapy

    NARCIS (Netherlands)

    Pas, R. van der; Esch, J.H. van; Bruin, C. de; Danser, A.H.; Pereira, A.M.; Zelissen, P.M.; Netea-Maier, R.; Sprij-Mooij, D.M.; Berg-Garrelds, I.M. van den; Schaik, R.H. van; Lamberts, S.W.J.; Meiracker, A.H. van den; Hofland, L.J.; Feelders, R.A.

    2014-01-01

    OBJECTIVE/METHODS: Cushing's disease (CD) is often accompanied by hypertension. CD can be treated surgically and, given the expression of somatostatin subtype 5 and dopamine 2 receptors by corticotroph pituitary adenomas, pharmacologically. Indeed, we recently observed that stepwise medical combinat

  9. A gender-dependent analysis of Cushing's disease in childhood: pre- and postoperative follow-up.

    Science.gov (United States)

    Libuit, Laura G; Karageorgiadis, Alexander S; Sinaii, Ninet; Nguyen May, Nina M; Keil, Margaret F; Lodish, Maya B; Stratakis, Constantine A

    2015-07-01

    To analyse gender differences in the clinical presentation and recovery of paediatric patients with Cushing's disease (CD) after transsphenoidal surgery (TSS). Indeed, gender differences between paediatric patients with CD during presentation, after TSS and postoperative recovery have not been adequately studied. Data were obtained and retrospectively analysed from clinical reports and biochemical tests at the time of presentation, 5-9 days after TSS and at the 6 and 12 months postoperative follow-up visits to determine hypothalamic-pituitary-adrenal axis (HPAA) recovery. Data from 102 paediatric patients (48 females, 54 males, mean age 12.9 ± 3.0) with CD who underwent TSS at the National Institute of Health (NIH) Clinical Center between 1997 and 2011. There was equal distribution of paediatric CD between males and females (53% vs 47%; n = 102, P = 0.484). Males were more likely than females to present with higher mean BMI Z-scores (2.2 ± 0.7 vs 1.9 ± 0.6, P = 0.0079), lower mean height Z-scores (-1.2 ± 1.3 vs -0.7 ± 1.1, P = 0.0467) and higher median plasma ACTH (12.2 vs 8.5 pmol/l; P = 0.0495). Females did not present more frequently with any single sign or symptom. No significant differences were found between males and females for CD cure rates 5-9 days after TSS (87.0% males vs 87.5% females, P = 1.0), long-term cure rates (86.5% vs 93.7%; n = 69; P = 0.4374) and HPAA recovery time (11.2 ± 2.5 vs 11.7 ± 2.5 months; n = 47; P = 0.1992). Paediatric CD is found to have equal distribution between males and females, but male patients present with elevated BMI and potentially shorter height and higher plasma ACTH. There is no significant difference in the cure rate or HPAA recovery time after TSS between males and females. Published 2014. This article is a U.S. Government work and is in the public domain in the USA.

  10. Bone and Joint Disorders in Acromegaly.

    Science.gov (United States)

    Claessen, Kim M J A; Mazziotti, Gherardo; Biermasz, Nienke R; Giustina, Andrea

    2016-01-01

    Acromegaly is a chronic, progressive disease caused by a growth hormone (GH)-producing pituitary adenoma, resulting in elevated GH and insulin-like growth factor 1 concentrations. Following appropriate therapy (surgery, radiotherapy and/or medical treatment), many systemic GH-induced comorbid conditions improve considerably. Unfortunately, despite biochemical control, acromegaly patients suffer from a high prevalence of late manifestations of transient GH excess, significantly impairing their quality of life. In this overview article, we summarize the pathophysiology, diagnosis, clinical picture, disease course and management of skeletal complications of acromegaly, focusing on vertebral fractures and arthropathy.

  11. Early descriptions of acromegaly and gigantism and their historical evolution as clinical entities.

    Science.gov (United States)

    Mammis, Antonios; Eloy, Jean Anderson; Liu, James K

    2010-10-01

    Giants have been a subject of fascination throughout history. Whereas descriptions of giants have existed in the lay literature for millennia, the first attempt at a medical description was published by Johannes Wier in 1567. However, it was Pierre Marie, in 1886, who established the term "acromegaly" for the first time and established a distinct clinical diagnosis with clear clinical descriptions in 2 patients with the characteristic presentation. Multiple autopsy findings revealed a consistent correlation between acromegaly and pituitary enlargement. In 1909, Harvey Cushing postulated a “hormone of growth" as the underlying pathophysiological trigger involved in pituitary hypersecretion in patients with acromegaly. This theory was supported by his observations of clinical remission in patients with acromegaly in whom he had performed hypophysectomy. In this paper, the authors present some of the early accounts of acromegaly and gigantism, and describe its historical evolution as a medical and surgical entity.

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

    Energy Technology Data Exchange (ETDEWEB)

    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).

  13. A consensus on the diagnosis and treatment of acromegaly complications

    OpenAIRE

    Melmed, S; Casanueva, F.F.; Klibanski, A; Bronstein, M. D.; Chanson, P.; Lamberts, S. W.; Strasburger, C. J.; Wass, J. A. H.; Giustina, A

    2012-01-01

    In March 2011, the Acromegaly Consensus Group met to revise and update the guidelines on the diagnosis and treatment of acromegaly complications. The meeting was sponsored by the Pituitary Society and the European Neuroendocrinology Association and included experts skilled in the management of acromegaly. Complications considered included cardiovascular, endocrine and metabolic, sleep apnea, bone diseases, and mortality. Outcomes in selected, related clinical conditions were also considered, ...

  14. Cardiovascular aspects in acromegaly: effects of treatment.

    Science.gov (United States)

    Lombardi, G; Colao, A; Ferone, D; Marzullo, P; Landi, M L; Longobardi, S; Iervolino, E; Cuocolo, A; Fazio, S; Merola, B; Sacca, L

    1996-08-01

    Patients with acromegaly have significant morbidity and mortality, associated with cardiovascular disease. Acromegaly is often complicated by other diseases such as diabetes mellitus, hypertension, and coronary artery disease, so the existence of acromegalic cardiomyopathy remains uncertain. Cardiac performance was investigated in patients with uncomplicated acromegaly. A subgroup of hypertensive acromegalics was also studied. In addition, the effects of chronic octreotide therapy or surgery on cardiac structure and function in acromegaly were studied. Twenty-six patients and 15 healthy controls underwent gated blood-pool cardiac scintigraphy and echocardiography at rest and during exercise. Echocardiography was repeated after 6 months of octreotide therapy (n = 11). Cardiac scintigraphy was repeated after 12 and 24 months of octreotide therapy (n = 10) or 12 to 24 months after surgery (n = 8). ECG, blood pressure, and heart rate were monitored during cardiac scintigraphy. Left ventricular mass (LVM) was calculated from the findings of the echocardiography. Serum growth hormone (GH) levels and plasma insulin-like growth factor-1 (IGF-1) levels were monitored. LVM index was significantly higher (P cardiac function were similar. Chronic octreotide decreased GH and IGF-1 levels and improved the structural abnormalities as measured by echocardiography. Chronic octreotide or surgery did not alter cardiac function parameters. Thus, important changes in cardiac structure and function occur in uncomplicated acromegaly, and improvements can be demonstrated after chronic octreotide therapy. Heart disease in acromegaly appears to be secondary to high circulating GH levels.

  15. Double, Synchronous Pituitary Adenomas Causing Acromegaly and Cushing’s Disease. A Case Report and Review of Literature

    OpenAIRE

    Zieliński, Grzegorz; Maksymowicz, Maria; Podgórski, Jan; Olszewski, Włodzimierz T

    2013-01-01

    Double pituitary adenomas are very rare and present up to 1 % of pituitary adenomas in unselected autopsy series and up to 2 % in large surgical series. We report a case of a 47-year-old man presented slight clinical features of acromegaly with 2 years duration. Endocrine evaluation confirmed active acromegaly and revealed adrenocorticotropin hormone-dependent hypercortisolemia. Preoperative magnetic resonance imaging of the pituitary demonstrated clearly separated double microadenomas with d...

  16. Ectopic Cushing syndrome

    Science.gov (United States)

    ... Cushing syndrome in which a tumor outside the pituitary gland produces a hormone called adrenocorticotropic hormone (ACTH). Causes Cushing syndrome is a disorder that occurs when your body has a higher ...

  17. Epidemiology of acromegaly in Ecuador.

    Science.gov (United States)

    López Gavilanez, Enrique; Guerrero Franco, Kempis; Solórzano Zambrano, Narcisa; Navarro Chávez, Manuel; López Estrella, Camilo; Vaca Burbano, Luis; Marriott Díaz, Eduardo

    2016-01-01

    To assess the epidemiology of acromegaly in the city of Guayaquil, Ecuador, and to compare our results to those reported in the literature. An analysis was made of retrospective and prospective data from all patients with acromegaly attending endocrinology clinics at the 4 main hospitals of the public health network of Guayaquil from January 2000 to December 2014. Age at diagnosis, estimated delay in diagnosis, imaging studies of pituitary gland, basal growth hormone (GH) level, GH after an oral glucose tolerance test (OGTT-GH), and serum levels of insulin-like growth factor 1 (IGF-1) were recorded. Incidence and prevalence of the disease were estimated using information from the 2010 census of population and housing. Forty-eight cases were recorded in the study period in 17 males (35.4%) and 31 females (64.5%); M/F ratio=1.8:1. Mean age at diagnosis was 47.3±16.8 years (range 18-86). Delay in diagnosis was 7.3±6.3 years (range 1-30). Mean age at diagnosis was 47.9±18.2 years in males and 46.3±15.8 years in females. Delay in diagnosis was 10.2±7.9 and 5.7±3.9 years in males and females, respectively. Prevalence of acromegaly is 18.7 cases per million inhabitants, and incidence of acromegaly 1.3 cases per million people per year. Acromegaly predominates in females, and is diagnosed in the fourth decade with a delay of approximately 8 years, usually even longer in males. Incidence and prevalence are lower than reported in international series. The disease is underdiagnosed and underreported in Ecuador. Copyright © 2016 SEEN. Publicado por Elsevier España, S.L.U. All rights reserved.

  18. Cutaneous findings in patients with acromegaly.

    Science.gov (United States)

    Akoglu, Gulsen; Metin, Ahmet; Emre, Selma; Ersoy, Reyhan; Cakir, Bekir

    2013-01-01

    Acromegaly is a systemic syndrome caused by overproduction of growth hormone. The syndrome affects cutaneous, endocrine, cardiovascular, skeletal, and respiratory systems. Cutaneous manifestations of acromegaly are various, usually being the first presenting findings of the disease. Forty-nine patients with acromegaly, followed-up at a tertiary referral hospital, underwent dermatological examination. There were 27 (55.1%) female and 22 (44.9%) male patients. The age at onset of the disease was older in females than males (P=0.045). Most patients had acral enlargements, large triangular nose, coarse face, thickened lower lip, and prognathism. Fourteen (28.6%) patients had multiple cherry angiomas, five (10.2%) had varicose veins in lower limbs, and two (4.1%) had psoriasis. In conclusion, a wide spectrum of cutaneous symptoms and features may be associated with acromegaly. Detailed dermatological examination of patients with acromegaly should be an essential component of systemic evaluation. Future prospective studies investigating the relationships between changes in skin signs, hormone levels, and response to treatments may help understand details of skin involvement in acromegaly.

  19. Prevalence of acromegaly in patients with symptoms of sleep apnea

    Science.gov (United States)

    Resmini, Eugenia; Sambo, Marcel; Blanco, Concepción; Calvo, Fernando; Pazos, Fernando; Fernández-Catalina, Pablo; Martínez de Icaya, Purificación; Páramo, Concepción; Fajardo, Carmen; Marazuela, Mónica; Álvarez-Escolá, Cristina; Díez, Juan Jose; Perea, Verónica

    2017-01-01

    Acromegaly is a rare disease with nonspecific symptoms with acral enlargement being almost universally present at diagnosis. The estimated prevalence is 40–125 cases/million but targeted universal screening studies have found a higher prevalence (about 10 fold). The aim of the ACROSAHS study was to investigate the prevalence of acromegaly and acromegaly comorbidities in patients with sleep apnea symptoms and acral enlargement. ACROSAHS was a Spanish prospective non-interventional epidemiological study in 13 Hospital sleep referral units. Facial and acral enlargement symptoms including: ring size and shoe size increase, tongue, lips and jaws enlargement, paresthesia or carpal tunnel syndrome and widening of tooth spaces, as well as other typical acromegaly comorbidities were recorded with a self-administered questionnaire of patients who attended a first visit for sleep apnea symptoms between 09/2013 and 07/2014. Serum insulin-like growth factor type 1 (IGF1) was measured in patients with ≥1 acral symptom to determine the prevalence of acromegaly. Of the 1557 patients enrolled, 1477 with complete data (72% male) were analyzed. 530 patients (36%) reported at least 1 acral enlargement symptom and were tested for IGF-1, 41 were above range, persisted in 7, and among those, 2 cases of acromegaly were diagnosed (prevalence of at least 1.35 cases/1000). Overall, 1019 patients (69%) had ≥2 acromegaly symptoms and should have been screened according to guidelines; moreover 373 patients (25%) had ≥1 symptom of acral enlargement plus ≥3 other acromegaly symptoms. In conclusion, in patients with sleep apnea symptoms and acral enlargement, we found an acromegaly prevalence of at least 1.35 cases per 1000 and a high prevalence of typical acromegaly symptoms. It is important that sleep specialists are aware of acromegaly symptoms to aid with acromegaly diagnosis. PMID:28898247

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

    Science.gov (United States)

    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

  1. Need for improved monitoring in patients with acromegaly

    Science.gov (United States)

    Silverstein, Julie M

    2015-01-01

    Acromegaly is a rare and insidious disease characterized by the overproduction of growth hormone (GH) and insulin-like growth factor 1 (IGF1) and is most commonly due to a pituitary adenoma. Patients with acromegaly who experience prolonged exposure to elevated levels of GH and IGF1 have an increased mortality risk and progressive worsening of disease-related comorbidities. Multimodal treatment with surgery, medical therapy, and radiotherapy provides biochemical control, defined by recent acromegaly clinical guidelines from the Endocrine Society as a reduction of GH levels to acromegaly, even those without clinical symptoms of disease, require long-term monitoring of GH and IGF1 levels if the benefits associated with biochemical control are to be maintained and the risk of developing recurrent disease is to be abated. However, suboptimal monitoring is common in patients with acromegaly, and this can have negative health effects due to delays in detection of recurrent disease and implementation of appropriate treatment. Because of the significant health consequences associated with prolonged exposure to elevated levels of GH and IGF1, optimal monitoring in patients with acromegaly is needed. This review article will discuss the biochemical assessments used for therapeutic monitoring in acromegaly, the importance of monitoring after surgery and medical therapy or radiotherapy, the consequences of suboptimal monitoring, and the need for improved monitoring algorithms for patients with acromegaly. PMID:26381160

  2. Cushing syndrome in pediatrics.

    Science.gov (United States)

    Stratakis, Constantine A

    2012-12-01

    Cushing syndrome is characterized by truncal obesity, growth deceleration, skin changes, muscle weakness, and hypertension. Cushing syndrome in childhood usually results from the exogenous administration of glucocorticoids. This article presents the causes and discusses the treatment of endogenous Cushing syndrome. It also discusses the clinical and molecular genetics of inherited forms of this syndrome. Cushing syndrome needs to be diagnosed and treated properly when first recognized; improper treatment can turn this otherwise completely curable disorder into a chronic ailment. Barriers to optimal care of a pediatric patient with Cushing syndrome are discussed. Published by Elsevier Inc.

  3. Endoscopic treatment of Cushing's disease via transsphenoidal approach%库欣病神经内镜下经蝶入路手术治疗

    Institute of Scientific and Technical Information of China (English)

    石海平; 罗坤; 周凯; 张庭荣

    2010-01-01

    @@ 库欣综合征(Cushing's syndrome, CS)又称皮质醇增多症,即高皮质醇血症及由其产生的一系列病理生理改变,是三种独立的病理综合征(垂体病变、肾上腺肿瘤、异位性癌肿)的统称.CS中约70%~90%是由库欣病( Cushing's disease, CD)即垂体肾上腺皮质激素(adrenocorticotropic hormone, ACTH)腺瘤或垂体ACTH细胞增生引起.内镜配套设备的完善、内镜操作技术的成熟,以及内镜自身的优越性,使得纯内镜下经蝶手术迅速在临床推广应用.

  4. Respiratory problems in acromegaly.

    Science.gov (United States)

    Murrant, N J; Gatland, D J

    1990-01-01

    Death from respiratory causes in acromegaly is three times more common than in the general population and is most often the result of upper airways obstruction, although less commonly pulmonary dysfunction and disturbance of the central nervous system may occur. These factors may be found alone or in combination. Despite several reports of laryngeal involvement, upper airway obstruction in acromegaly is usually regarded as being due to macroglossia and pharyngeal soft tissue hypertrophy. We present four cases of acromegaly in which tracheostomy was required for laryngeal obstruction, with a review of the literature concerning the nature of respiratory problems in acromegaly.

  5. The problem of unrecognized acromegaly: surgeries patients undergo prior to diagnosis of acromegaly.

    Science.gov (United States)

    Keskin, F E; Yetkin, D O; Ozkaya, H M; Haliloglu, O; Sadri, S; Gazioglu, N; Tanrıover, N; Ak, H; Hatipoglu, E; Kadıoglu, P

    2015-06-01

    To reveal the variety of symptoms experienced by patients before acromegaly diagnosis and to emphasize unneeded surgeries that patients undergo related to acromegaly prior to diagnosis of the disease. In total, 490 consecutive adult patients with acromegaly who were treated at our institution between 1998 and 2014 were included in this cross-sectional study, of which 313 could be contacted. Participants were questioned about their complaints at initial consultation and at the time of diagnosis, the first medical professional who they consulted, interval between onset and diagnosis, and surgeries they had undergone. This study included 313 participants, of whom 181 were women. The mean age was 48.8 ± 12.0 years. Patients most frequently presented with acral growth of hands and feet (32.6%) and headache (26.2%). Internists were the medical specialists who were most frequently first consulted (29.4%) then neurosurgeons (11.8%). Acromegaly was generally diagnosed by endocrinologists (55%), followed by neurosurgeons (23%). The median elapsed period prior to diagnosis was 24 months, interquartile range 6.0-48.0 months. Some 45.7% had undergone surgery; 35.2% were related to acromegaly symptoms: head and throat surgery (12.8%), nose surgery (9.3%), thyroidectomy (6.4%), carpal tunnel surgery (4.8%). The delay period for patients who had an operation prior to acromegaly diagnosis was significantly longer than for those who had no operations (p Acromegaly patients mostly present to internal medicine professionals. Surgeries related to acromegaly complications and symptoms before diagnosis cause a long delay period before diagnosis. Medical staff must be more aware of the clinical aspects of acromegaly.

  6. [Current views on etiology, pathophysiology and clinical manifestations of acromegaly].

    Science.gov (United States)

    Krysiak, Robert; Okopień, Bogusław; Marek, Bogdan

    2012-02-01

    Acromegaly is a relatively rare disease caused by excess secretion of growth hormone, usually from a pituitary somatotrope adenoma. Most of the tumors causing acromegaly are sporadic but in some cases the disease results from the presence of an inherited endocrine syndrome including type 1 multiple endocrine neoplasia, McCune-Albright syndrome and Carney complex. Because of the slow evolution of signs and symptoms, acromegaly can be a diagnostic challenge. The disease is characterised by significantly increased morbidity and mortality, both of which are secondary to the involvement of multiple organ systems. This prompts the need for the rapid and accurate recognition and management of this disorder. Acromegaly is diagnosed on the basis of clinical picture, hormonal assays and radiologic imaging of the pituitary gland. This article reviews the etiology, histopathological picture, clinical features and current diagnostic strategies in acromegaly and prognosis in this disorder.

  7. Prevalence of diabetes mellitus in patients with acromegaly.

    Science.gov (United States)

    Dreval, A V; Trigolosova, I V; Misnikova, I V; Kovalyova, Y A; Tishenina, R S; Barsukov, I A; Vinogradova, A V; Wolffenbuttel, B H R

    2014-01-01

    Early carbohydrate metabolism disorders (ECMDs) and diabetes mellitus (DM) are frequently associated with acromegaly. We aimed to assess the prevalence of ECMDs in patients with acromegaly and to compare the results with those in adults without acromegaly using two population-based epidemiologic surveys. We evaluated 97 patients with acromegaly in several phases of their disease (mean age, 56 years and estimated duration of acromegaly, 12.5 years). An oral glucose tolerance test was done in those not yet diagnosed with DM to reveal asymptomatic DM or ECMDs (impaired glucose tolerance+impaired fasting glucose). Comparisons were made between patients with acromegaly and participants from the general adult population (n=435) and an adult population with multiple type 2 diabetes risk factors (n=314), matched for gender, age and BMI. DM was diagnosed in 51 patients with acromegaly (52.5%) and 14.3% of the general population (Pacromegaly than in the general population and in the high-risk group; only 22% of patients with acromegaly were normoglycaemic. The prevalence of newly diagnosed ECMDs or DM was 1.3-1.5 times higher in patients with acromegaly compared with the high-risk group. Patients with acromegaly having ECMDs or DM were older, more obese and had longer disease duration and higher IGF1 levels (Z-score). Logistic regression showed that the severity of glucose derangement was predicted by age, BMI and IGF1 levels. In patients with acromegaly, the prevalence of DM and ECMDs considerably exceeds that of the general population and of a high-risk group, and development of DM depends on age, BMI and IGF1 levels.

  8. Long term follow-up of patients with Cushing's disease treated by interstitial irradiation

    Energy Technology Data Exchange (ETDEWEB)

    Sandler, L.M.; Richards, N.T.; Carr, D.H.; Mashiter, K.; Joplin, G.F.

    1987-09-01

    The first 86 patients with Cushing's disease treated with interstitial irradiation (by needle implantation) as the sole therapy were reviewed. In the 82 patients who were reassessed 1 yr after treatment 63 (77%) achieved remission. This study comprises the outcome and complications in the 54 patients who had a remission and whom we were able to follow. The follow-up period ranged from 3-26 yr (mean, 10.5) from the time of remission. No instance of clinical or radiological relapse has occurred. Of these 54 patients, yttrium-90 alone was used in 32, of whom 12 (37%) required corticosteroid or T4 replacement therapy in a mean time of 3.5 months; in 7 of these 12 we elected to give an ablative dose. Gold-198 alone was used in 15 patients, of whom 7 (47%) developed hypopituitarism in a mean time of 76 months. Both isotopes were used in 7 patients. A diurnal serum cortisol rhythm was found in 28 of the 31 patients who were not receiving corticosteroid therapy. In 5 of the 7 patients with an initially abnormal pituitary fossa, serial radiological studies revealed remodelling in 3. There have been no complications in the last 17 years. Pituitary implantation with yttrium-90 is an effective alternative to transsphenoidal hypophysectomy, with a high remission rate, no recurrence (as yet), no operative complications, and avoidance of hormone replacement in the majority.

  9. Growth hormone, growth factors, and acromegaly

    Energy Technology Data Exchange (ETDEWEB)

    Ludecke, D.K.; Tolis, G.T.

    1987-01-01

    This book contains five sections, each consisting of several papers. The section headings are: Biochemistry and Physiology of GH and Growth Factors, Pathology of Acromegaly, Clinical Endocrinology of Acromegaly, Nonsurgical Therapy of Acromegaly, and Surgical Therapy of Acromegaly.

  10. Pregnancy and acromegaly

    NARCIS (Netherlands)

    A. Muhammad (Ammar); S.J.C.M.M. Neggers (Bas); A-J. van der Lely (Aart-Jan)

    2016-01-01

    textabstractIntroduction: Acromegaly is a rare disorder in which, due to the high incidence of secondary hypogonadism, pregnancies are relatively rare. However, some women with acromegaly do get pregnant, which brings along questions about medication, complications and follow-up. This review tries

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

    Science.gov (United States)

    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

  12. Cardiovascular complications in acromegaly: methods of assessment.

    Science.gov (United States)

    Vitale, G; Pivonello, R; Galderisi, M; D'Errico, A; Spinelli, L; Lupoli, G; Lombardi, G; Colao, A

    2001-09-01

    Cardiac involvement is common in acromegaly. Evidence for cardiac hypertrophy, dilation and diastolic filling abnormalities has been widely reported in literature. Generally, ventricular hypertrophy is revealed by echocardiography but early data referred increased cardiac size by standard X-ray. Besides, echocardiography investigates cardiac function and value disease. There are new technologic advances in ultrasonic imaging. Pulsed Tissue Doppler is a new non-invasive ultrasound tool which extends Doppler applications beyond the analysis of intra-cardiac flow velocities until the quantitative assessment of the regional myocardial left ventricular wall motion, measuring directly velocities and time intervals of myocardium. The radionuclide techniques permit to study better the cardiac performance. In fact, diastolic as well as systolic function can be assessed at rest and at peak exercise by equilibrium radionuclide angiography. This method has a main advantage of providing direct evaluation of ventricular function, being operator independent. Coronary artery disease has been poorly studied mainly because of the necessity to perform invasive procedures. Only a few cases have been reported with heart failure study by coronarography and having alterations of perfusion which ameliorated after somatostatin analog treatment. More recently, a few data have been presented using perfusional scintigraphy in acromegaly, even if coronary artery disease does not seem very frequent in acromegaly. Doppler analysis of carotid arteries can be also performed to investigate atherosclerosis: however, patients with active acromegaly have endothelial dysfunction more than clear-cut atherosclerotic plaques. In conclusion, careful assessments of cardiac function, morphology and activity need in patients with acromegaly.

  13. [Epidemiology of acromegaly in Spain].

    Science.gov (United States)

    Sesmilo, Gemma

    2013-10-01

    Epidemiology of acromegaly in Spain does not differ from that reported in other published series. Prevalence rate is approximately 60 cases per million, peak incidence occurs in middle age, more women are affected (61%), and there is a substantial delay between occurrence of the first symptoms and diagnosis. Studies REA (Spanish Acromegaly Registry) and OASIS analyzed the epidemiology, clinical characteristics, and management of the disease in Spain. Surgery, performed in more than 80% of patients, has been (and continues to be) the main treatment for the past four decades. In the past decade, however, more patients have received somatostatin analogs (SSAs) as first-line treatment. Use of radiation therapy has significantly decreased in recent decades. Somatostatin analogs (SSAs) are the most commonly used drugs, administered to 85% of patients; however, only 12%-15% continue on drug treatment alone. The surgical remission rate was 38.4% in the last decade, with a significant improvement over decades. Preoperative treatment with SSAs has no influence on surgical cure rates. Second-line therapies used after surgical failure in the past decade included SSAs in 49% of patients, repeat surgery in 27%, radiotherapy in 11%, pegvisomant in 15%, and dopamine agonists in 5%. Mean cost of acromegaly treatment was 9.668€ (data estimated in 2009 and adjusted in 2010), of which 71% was due to the cost of SSAs. Patients treated with pegvisomant have a more aggressive form of the disease and higher comorbidity rates.

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

    Science.gov (United States)

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

    2017-05-01

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

  15. Complications of acromegaly: cardiovascular, respiratory and metabolic comorbidities.

    Science.gov (United States)

    Pivonello, Rosario; Auriemma, Renata S; Grasso, Ludovica F S; Pivonello, Claudia; Simeoli, Chiara; Patalano, Roberta; Galdiero, Mariano; Colao, Annamaria

    2017-02-01

    Acromegaly is associated with an enhanced mortality, with cardiovascular and respiratory complications representing not only the most frequent comorbidities but also two of the main causes of deaths, whereas a minor role is played by metabolic complications, and particularly diabetes mellitus. The most prevalent cardiovascular complications of acromegaly include a cardiomyopathy, characterized by cardiac hypertrophy and diastolic and systolic dysfunction together with arterial hypertension, cardiac rhythm disorders and valve diseases, as well as vascular endothelial dysfunction. Biochemical control of acromegaly significantly improves cardiovascular disease, albeit completely recovering to normal mainly in young patients with short disease duration. Respiratory complications, represented mainly by sleep-breathing disorders, particularly sleep apnea, and respiratory insufficiency, frequently occur at the early stage of the disease and, although their severity decreases with disease control, this improvement does not often change the indication for a specific therapy directed to improve respiratory function. Metabolic complications, including glucose and lipid disorders, are variably reported in acromegaly. Treatments of acromegaly may influence glucose metabolism, and the presence of diabetes mellitus in acromegaly may affect the choice of treatments, so that glucose homeostasis is worth being monitored during the entire course of the disease. Early diagnosis and prompt treatment of acromegaly, aimed at obtaining a strict control of hormone excess, are the best strategy to limit the development or reverse the complications and prevent the premature mortality.

  16. Epidemiology of acromegaly.

    Science.gov (United States)

    Holdaway, I M; Rajasoorya, C

    1999-06-01

    Acromegaly is a consequence of chronic growth hormone (GH) excess, due in the majority of cases to a GH-secreting pituitary adenoma, and occurring with a population prevalence of 60 per million and an incidence of 3-4 per million per year. Males and females appear to be equally affected with an average age of presentation of 44 years. Younger patients may have more aggressive tumours and higher GH concentrations. There is co-existent hyperprolactinaemia in about one third of cases, and a variable proportion of [figure: see text] tumours appear to have activating mutations of the gsp gene or other genetic abnormalities. Acute complications such as carpal tunnel syndrome, sweating and obstructive sleep apnoea are usually readily reversible with treatment of the condition, but chronic complications such as hypertension, diabetes and heart disease are less readily corrected and post-treatment GH levels of acromegaly leading to increased morbidity and mortality from the disorder, with observed-to-expected mortality ratios ranging from 1.6-3.3 and only approaching unity in those with growth hormone levels < 2.5 ug/L following treatment. Prognostic factors include in some studies the presence of diabetes and [table: see text] hypertension prior to diagnosis as well as measures of exposure to excessive growth hormone derived from the product of preoperative serum GH and the time from first symptoms to treatment. Overall, however, the most important prognostic variable appears to be the serum GH concentration achieved by treatment, with an increasing consensus that this needs to be < 2.5 ug/L (5 mU/L) to achieve cure of the condition.

  17. Another 'Cushing ulcer'.

    Science.gov (United States)

    Hoshino, Chisho; Satoh, Noriyuki; Narita, Masashi; Kikuchi, Akio; Inoue, Minoru

    2011-04-09

    The authors describe the case of a 39-year-old man who presented to our hospital with easy fatigability and malaise. On physical examination, hypertension was noted without any cushingoid appearance. Laboratory testing revealed normochromic-normocytic anaemia with positive results of occult blood in the stool, hyperglycaemia and hypokalemia. Upper endoscopy revealed active gastric ulcer with Helicobacter pylori infection, likely causing gastrointestinal bleeding. Endocrine examinations showed that both serum adrenocorticotropic hormone and cortisol were elevated with loss of diurnal variation. A diagnosis of Cushing's disease secondary to pituitary adenoma was made as results of brain MRI and blood sampling from inferior petrosal sinus. In a patient with peptic ulcer disease, physician should be alert to the possible endocrine background.

  18. Assessment of lung function in a large cohort of patients with acromegaly.

    Science.gov (United States)

    Störmann, Sylvère; Gutt, Bodo; Roemmler-Zehrer, Josefine; Bidlingmaier, Martin; Huber, Rudolf M; Schopohl, Jochen; Angstwurm, Matthias W

    2017-07-01

    Acromegaly is associated with increased mortality due to respiratory disease. To date, lung function in patients with acromegaly has only been assessed in small studies, with contradicting results. We assessed lung function parameters in a large cohort of patients with acromegaly. Lung function of acromegaly patients was prospectively assessed using spirometry, blood gas analysis and body plethysmography. Biochemical indicators of acromegaly were assessed through measurement of growth hormone and IGF-I levels. This study was performed at the endocrinology outpatient clinic of a tertiary referral center in Germany. We prospectively tested lung function of 109 acromegaly patients (53 male, 56 female; aged 24-82 years; 80 with active acromegaly) without severe acute or chronic pulmonary disease. We compared lung volume, air flow, airway resistance and blood gases to normative data. Acromegaly patients had greater lung volumes (maximal vital capacity, intra-thoracic gas volume and residual volume: P acromegaly. Female patients had significantly altered lung function in terms of subclinical airway obstruction. In our cross-sectional analysis of lung function in 109 patients with acromegaly, lung volumes were increased compared to healthy controls. Additionally, female patients showed signs of subclinical airway obstruction. There was no difference between patients with active acromegaly compared with patients biochemically in remission. © 2017 European Society of Endocrinology.

  19. Combination therapy for Cushing's disease: effectiveness of two schedules of treatment: should we start with cabergoline or ketoconazole?

    Science.gov (United States)

    Barbot, M; Albiger, N; Ceccato, F; Zilio, M; Frigo, A C; Denaro, L; Mantero, F; Scaroni, C

    2014-04-01

    Cushing's disease (CD) is associated with increased morbidity and mortality. Until now, no medical treatment has been shown to be totally satisfactory when administrated alone. This study aimed to assess the effectiveness of cabergoline with added ketoconazole and of the same combination in reverse, using urinary free cortisol (UFC) and late night salivary cortisol (LNSC) levels as biochemical markers of the treatments' efficacy in CD patients. A prospective analysis conducted on 14 patients (f/m = 12/2; median age 52, range 33-70 years) divided into two groups: 6 patients initially treated with cabergoline for 4-6 months (rising from 0.5-1 mg/week up to 3.0 mg/week), after which ketoconazole was added (group A); and 8 patients first took ketoconazole alone for 4-6 months (rising from 200 mg/day to 600 mg/day), then cabergoline was added (group B). Patients were compared with 14 age-matched patients in prolonged remission after effective neurosurgery for CD. The combination therapy led to UFC normalization in 79 % of patients with no differences between the groups; only one patient failed to respond at all. Neither drug succeeded in controlling the disease when taken alone. LNSC dropped when compared to baseline levels, but not to a significant degree (p = 0.06), and it remained significantly higher than in controls (p = 0.0006). Associating cabergoline with ketoconazole may represent an effective second-line treatment, achieving a satisfactory reduction in UFC levels and clinical improvement. Although the combined treatment lowered patients' LNSC levels, they remained higher than normal, indicating a persistent subclinical hypercortisolism; the implications of this condition need to be considered. No differences emerged between the two treatment schedules.

  20. [Acromegaly: multifaceted clinical presentation of a rare disease of the elderly. Report of two cases with long-term follow-up].

    Science.gov (United States)

    Foppiani, Luca; Del Monte, Patrizia; Ruelle, Antonio; Marugo, Alessandro; Bernasconi, Donatella

    2006-04-01

    We report two cases of acromegaly in elderly patients. Both patients had markedly invasive GH-secreting macroadenomas, which caused hugely increased circulating GH levels (over 90 ng/ml). The first patient, 79 year-old, presented with goitre and severe osteoarthrosis, refused surgery and was treated with various somatostatin analogues (ultimately accompanied by cabergoline), without satisfactory control of the disease. The second patient, 67-year-old, presented with symptoms secondary to hypopituitarism, which had been previously misdiagnosed. These symptoms resolved with the appropriate substitutive therapy, which led to a significant improvement in her condition. However, two transphenoidal operations, radiotherapy and long-term somatostatin agonist therapy were required to control GH hypersecretion satisfactorily. The authors wish to underline that acromegaly is a rare but not negligible disorder in the elderly, which can affect the whole body functions and cause severe morbidities. In the two cases presented somatostatin agonists alone were not able to control the tumoral hypersecretion adequately. The prompt discovery (usually through a simple clinical evaluation) of this disease in the elderly, confirmed by hormonal and morphological evaluation, together with a multidisciplinary (medical, surgical, radiotherapeutic) approach can improve their quality of life and increase life expectancy.

  1. [Acromegaly features in the aging population].

    Science.gov (United States)

    Anoun, Nadia; El Ouahabi, Hanan

    2017-01-01

    Somatotroph adenomas are rare in the aging population. Diagnosis of somatotroph adenomas is often long delayed and they are characterized by atypical clinical picture. Their diagnostic criteria are similar to those used for younger patients. Surgery, if possible, is the treatment of choice for acromegaly in the elderly. Somatostatin analogues have shown to be effective in these patients. Prognosis is inversely correlated with patient's age, duration of disease and last GH level under treatment. Beside evolution of disease, age is a major determinant of mortality. We report three cases of elderly patients with acromegaly aged 75, 70 and 66 years respectively with a literature review.

  2. Medical Therapy of Acromegaly

    Directory of Open Access Journals (Sweden)

    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.

  3. Pasireotide for the treatment of acromegaly.

    Science.gov (United States)

    Wildemberg, Luiz Eduardo; Gadelha, Mônica R

    2016-01-01

    Acromegaly is a chronic disease with high morbidity and enhanced mortality if left untreated. Treatment options include surgery, medical therapy (somatostatin analogues (SA), dopamine agonists (DA) and growth hormone receptor antagonists) and radiotherapy. Despite these treatment options, "real-life" studies have shown that approximately 50% of patients are not controlled. In this scenario, a next-generation SA, pasireotide, has recently been approved for the treatment of acromegaly. 1) pasireotide's pharmacokinetics and pharmacodynamics; 2) pasireotide's anti-secretory and anti-proliferative effects, from preclinical studies up to phase III clinical trials; and 3) the adverse effects of pasireotide, focusing on hyperglycemia; 4) biomarkers of response to SA treatment. surgery is the primary treatment for most patients with acromegaly; however, approximately half of them will need adjuvant therapy. At present, the decision of this adjuvant treatment is made on a "trial-and-error" fashion. Nevertheless, in recent years, efforts have been made to establish biomarkers for the response to drugs involved in the treatment of acromegaly, which will change the treatment of acromegaly towards a more personalized therapeutic decision-making process. In the near future, the establishment of pasireotide response biomarkers will allow us to identify good candidates for first-line medical monotherapy with pasireotide.

  4. Negative correlation between tumour size and cortisol/ACTH ratios in patients with Cushing's disease harbouring microadenomas or macroadenomas.

    Science.gov (United States)

    Machado, M C; Alcantara, A E E; Pereira, A C L; Cescato, V A S; Castro Musolino, N R; de Mendonça, B B; Bronstein, M D; Fragoso, M C B V

    2016-12-01

    Pituitary macroadenomas (MACs) represent 10-30 % of Cushing's disease (CD) cases. The aim of this study was to report the clinical, laboratorial and imaging features and postsurgical outcomes of microadenoma (MIC) and MAC patients. Retrospective study with 317 CD patients (median 32 years old, range 9-71 years) admitted between 1990 and 2014, 74 (23.3 %) of whom had MAC. Hirsutism, plethora facial, muscular weakness and muscular atrophy were more frequent in the MIC patients. Nephrolithiasis, osteopenia, hyperprolactinaemia and galactorrhoea were more prevalent in MAC patients. The morning serum cortisol (Fs), nocturnal salivary cortisol (NSC), nocturnal Fs (Fs 2400 h), low- and high-dose dexamethasone suppression test results and CRH and desmopressin test results were similar between the subgroups. MIC patients showed higher urinary cortisol at 24 h (UC), and MAC patients presented higher ACTH levels but lower Fs/ACTH, Fs 2400 h/ACTH, NSC/ACTH and UC/ACTH ratios. There were negative correlations of tumour size with Fs/ACTH, Fs 2400 h/ACTH, NSC/ACTH and UC/ACTH ratios. Overall, the postsurgical remission and recurrence rates were similar between MIC and MAC. However, patients in remission (MIC + MAC) showed smaller tumour diameters and a lower prevalence of invasion and extension on MRI. Despite exhibiting higher plasma ACTH levels, CD patients with MAC presented lower cortisol/ACTH ratios than did patients with MIC, with a negative correlation between tumour size and cortisol/ACTH ratios. The overall postsurgical remission and recurrence rates were similar between MIC and MAC patients, with those with larger and/or invasive tumours showing a lower remission rate.

  5. Implementing a screening program for acromegaly in Latin America: necessity versus feasibility

    OpenAIRE

    Danilowicz, Karina; Day, Patricia Fainstein; Marcos P. Manavela; Herrera, Carlos Javier; Deheza, María Laura; Isaac, Gabriel; Juri, Ariel; Katz, Debora; Oscar D. Bruno

    2016-01-01

    Introduction Acromegaly is a rare disease with a large burden due its associated comorbidities and the life-long management required. Since the occurrence and severity of associated complications are related to length of exposure to the excess growth hormone seen in acromegaly, early diagnosis is imperative. The delay in diagnosis, however, can be long, and may be the result of a lack of disease awareness and screening programs. Since acromegaly is an uncommon disease, finding ways to increas...

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

    Energy Technology Data Exchange (ETDEWEB)

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

    1981-09-01

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

  7. Diabetes in Patients With Acromegaly.

    Science.gov (United States)

    Hannon, A M; Thompson, C J; Sherlock, M

    2017-02-01

    Acromegaly is a clinical syndrome which results from growth hormone excess. Uncontrolled acromegaly is associated with cardiovascular mortality, due to an excess of risk factors including diabetes mellitus, hypertension and cardiomegaly. Diabetes mellitus is a frequent complication of acromegaly with a prevalence of 12-37%. This review will provide an overview of a number of aspects of diabetes mellitus and glucose intolerance in acromegaly including the following: 1. Epidemiology and pathophysiology of abnormalities of glucose homeostasis 2. The impact of different management options for acromegaly on glucose homeostasis 3. The management options for diabetes mellitus in patients with acromegaly RECENT FINDINGS: Growth hormone and IGF-1 have complex effects on glucose metabolism. Insulin resistance, hyperinsulinaemia and increased gluconeogenesis combine to produce a metabolic milieu which leads to the development of diabetes in acromegaly. Treatment of acromegaly should ameliorate abnormalities of glucose metabolism, due to reversal of insulin resistance and a reduction in gluconeogenesis. Recent advances in medical therapy of acromegaly have varying impacts on glucose homeostasis. These adverse effects influence management choices in patients with acromegaly who also have diabetes mellitus or glucose intolerance. The underlying mechanisms of disorders of glucose metabolism in patients with acromegaly are complex. The aim of treatment of acromegaly is normalisation of GH/IGF-1 with reduction of co-morbidities. The choice of therapy for acromegaly should consider the impact of therapy on several factors including glucose metabolism.

  8. Acromegaly: Beyond surgery

    OpenAIRE

    Gaya Thanabalasingham; Grossman, Ashley B.

    2013-01-01

    Acromegaly is characterized by chronic, excess secretion of growth hormone (GH) from a pituitary adenoma, and elevated hepatic insulin-like growth factor 1 (IGF-1) levels. Significant progress has been made in the development of medical therapies to achieve biochemical and symptomatic control in acromegaly. In this review we discuss the three currently available medical therapies, which include somatostatin analogs, dopamine agonists and pegvisomant. We describe a step-wise approach in which ...

  9. Diabetic retinopathy in acromegaly

    OpenAIRE

    Said Azzoug; Farida Chentli

    2014-01-01

    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...

  10. Health-related quality of life in pituitary diseases.

    Science.gov (United States)

    Crespo, Iris; Valassi, Elena; Santos, Alicia; Webb, Susan M

    2015-03-01

    In the last 15 years, worse health-related quality of life (QoL) has been reported in patients with pituitary diseases compared with healthy individuals. Different QoL questionnaires have shown incomplete physical and psychological recovery after therapy. Residual impairments often affect QoL even long-term after successful treatment of pituitary adenomas. In this article, knowledge of factors that affect QoL in pituitary diseases is reviewed. The focus is on 5 pituitary diseases: Cushing syndrome, acromegaly, prolactinomas, nonfunctioning pituitary adenomas, and hypopituitarism.

  11. Recovery of the hypothalamic-pituitary-adrenal axis in children and adolescents after surgical cure of Cushing's disease.

    Science.gov (United States)

    Lodish, Maya; Dunn, Somya Verma; Sinaii, Ninet; Keil, Margaret F; Stratakis, Constantine A

    2012-05-01

    Recovery of the hypothalamic-pituitary-adrenal axis (HPAA) after transsphenoidal surgery (TSS) for Cushing's disease (CD) in children has not been adequately studied. Our objective was to assess time to recovery of the HPAA after TSS in children with CD. This was a case series at the National Institutes of Health Clinical Center. Fifty-seven patients with CD (6-18 yr, mean 13.0 ± 3.1 yr) given a standard regimen of glucocorticoid tapering after TSS were studied out of a total of 73 recruited. ACTH (250 μg) stimulation tests were administered at approximately 6-month intervals for up to 36 months. Age, sex, pubertal status, body mass index, length of disease, midnight cortisol, and urinary free cortisol at diagnosis were analyzed for effects on recovery. The main outcome measure was complete recovery of the HPAA as defined by a cortisol level of at least 18 μg/dl in response to 250 μg ACTH. Full recovery was reached by 43 (75.4%) of 57 patients, with 29 of the 43 (67.4%) and 41 of the 43 (95.3%) recovering by 12 and 18 months, respectively. The overall mean time to recovery was 12.6 ± 3.3 months. Kaplan-Meier survivor function estimated a 50% chance of recovering by 12 months after TSS and 75% chance of recovering within 14 months. By receiver operating characteristic curve assessment, the cutoff of at least 10-11 μg/dl of cortisol as the peak of ACTH stimulation testing at 6 months after TSS yielded the highest sensitivity (70-80%) and specificity (64-73%) to predict full recovery of the HPAA at 12 months. Two of the four patients that recovered fully within 6 months had recurrent CD. Although this is not a randomized study, we present our standardized tapering regimen for glucocorticoid replacement after TSS that led to recovery of the HPAA in most patients within the first postoperative year. Multiple factors may affect this process, but an early recovery may indicate disease recurrence.

  12. LIMITATIONS OF CURRENT APPROACHES FOR THE TREATMENT OF ACROMEGALY.

    Science.gov (United States)

    Shanik, Michael H

    2016-02-01

    Acromegaly is a rare disease characterized by hypersecretion of growth hormone (GH), typically from a benign pituitary somatotroph adenoma, that leads to subsequent hypersecretion of insulin-like growth factor 1 (IGF-1). Patients with acromegaly have an increased risk of mortality and progressive worsening of comorbidities. Surgery, medical therapy, and radiotherapy are currently available treatment approaches for patients with acromegaly, with overall therapeutic goals of lowering GH levels and achieving normal IGF-1 levels, reducing tumor size, improving comorbidities, and minimizing mortality risk. Although surgery can lead to biochemical remission in some patients with acromegaly, many patients will continue to have uncontrolled disease and require additional treatment. We reviewed recently published reports and present a summary of the safety and efficacy of current treatment modalities for patients with acromegaly. A substantial proportion of patients who receive medical therapy or radiotherapy will have persistently elevated GH and/or IGF-1. Because of the serious health consequences of continued elevation of GH and IGF-1, there is a need to improve therapeutic approaches to optimize biochemical control, particularly in high-need patient populations for whom current treatment options provide limited benefit. This review discusses current treatment options for patients with acromegaly, limitations associated with each treatment approach, and areas within the current treatment algorithm, as well as patient populations for which improved therapeutic options are needed. Novel agents in development were also highlighted, which have the potential to improve management of patients with uncontrolled or persistent acromegaly.

  13. [The importance of early diagnosis in acromegaly].

    Science.gov (United States)

    Valkusz, Zsuzsanna; Tóth, Miklós; Boda, Judit; Nagy, Endre; Julesz, János

    2011-05-01

    The authors review the historical and epidemiological aspects, clinical features and complications of acromegaly while emphasizing the importance of the early diagnosis and treatment. Acromegaly is a rare and mostly sporadic disorder due to excessive production of growth hormone. It is characterized by progressive somatic disfigurement (mainly involving the face and extremities) and systemic manifestations. The prevalence is estimated between 40 and 125 cases/million. Generally, it is diagnosed in middle-aged adults (mean age 40 years, men and women equally affected). Due to its insidious onset and slow progression, acromegaly is often diagnosed 7 to more than 10 years after its onset. The disease has cardiovascular, rheumatological, respiratory and metabolic consequences which highly determine its prognosis. Acromegaly is associated with a number of complications resulting in a two- or four-fold increase of mortality and a decrease of life expectancy by about 10 years. The major causes of death include cardiovascular and cerebrovascular events, respiratory diseases and malignancies. The duration of the disease before the introduction of effective therapy may be a major predictor of increased mortality mainly due to complications . The early diagnosis is important for timely commencement of treatment and for prevention of serious complications of the disease.

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

    OpenAIRE

    Ceccato F; Scaroni C; Boscaro M

    2015-01-01

    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 ta...

  15. Harvey Cushing's Canadian connections.

    Science.gov (United States)

    Feindel, William

    2003-01-01

    During his surgical career between 1896 and 1934, Harvey Cushing made eight visits to Canada. He had a broad impact on Canadian medicine and neurosurgery. Cushing's students Wilder Penfield and Kenneth McKenzie became outstanding leaders of the two major centers in Canada for neurosurgical treatment and training. On his first trip to Canada, shortly after completing his surgical internship in August 1896, Cushing traveled with members of his family through the Maritime Provinces and visited hospitals in Quebec and Montreal. Eight years later, in February 1904, as a successful young neurosurgeon at the Johns Hopkins Hospital, he reported to the Montreal Medico-Chirurgical Society on his surgical experience in 20 cases of removal of the trigeminal ganglion for neuralgia. In 1922, as the Charles Mickle Lecturer at the University of Toronto, Cushing assigned his honorarium of $1000 to support a neurosurgical fellowship at Harvard. This was awarded to McKenzie, then a general practitioner, for a year's training with Cushing in 1922-1923. McKenzie returned to initiate the neurosurgical services at the Toronto General Hospital, where he developed into a master surgeon and teacher. On Cushing's second visit to McGill University in October 1922, he and Sir Charles Sherrington inaugurated the new Biology Building of McGill's Medical School, marking the first stage of a Rockefeller-McGill program of modernization. In May 1929, Cushing attended the dedication of the Osler Library at McGill. In September 1934, responding to the invitation of Penfield, Cushing presented a Foundation Lecture-one of his finest addresses on the philosophy of neurosurgery-at the opening of the Montreal Neurological Institute. On that same trip, Cushing's revisit to McGill's Osler Library convinced him to turn over his own treasure of historical books to Yale University.

  16. The Liege Acromegaly Survey (LAS): a new software tool for the study of acromegaly.

    Science.gov (United States)

    Petrossians, Patrick; Tichomirowa, Maria A; Stevenaert, Achile; Martin, Didier; Daly, Adrian F; Beckers, Albert

    2012-06-01

    Acromegaly is a chronic rare disease associated with negative pathological effects on multiple systems and organs. We designed a new informatics tool to study data from patients with acromegaly, the Liege Acromegaly Survey (LAS). This relational database permits the inclusion of anonymous historical and prospective data on patients and includes pathophysiology, clinical features, responses to therapy and long term outcomes of acromegaly. We deployed the LAS in a validation study at a single center in order to study the characteristics of patients with acromegaly diagnosed at our center from 1970-2011. A total of 290 patients with acromegaly were included (147 males and 143 females). There was a linear relationship between age at diagnosis and the date of diagnosis, indicating that older patients are being diagnosed with acromegaly more frequently. A majority presented with macroadenomas (77.5%) and the median diameter was 14 mm. Patients with macroadenomas were significantly younger than patients with microadenomas (P=0.01). GH values at diagnosis decreased with the age of the patients (P=0.01) and there was a correlation between GH values and tumor size at diagnosis (P=0.02). No correlation existed between insulin-like growth factor 1 (IGF-1) levels and tumor characteristics. The prevalence of diabetes was 21.4% in this population and 41.0% had hypertension. The presence of hypertension and diabetes were significantly associated with one another (P<0.001). There was a linear relation between initial GH and IGF-1 levels at diagnosis and those obtained during SSA analog treatment and the lowest GH and IGF-1 values following SSA therapy were obtained in older patients (GH: P<0.001; IGF-1: P<0.001). The LAS is a new relational database that is feasible to use in the clinical research setting and permits ready pooling of anonymous patient data from multiple study sites to undertake robust statistical analyses of clinical and therapeutic characteristics.

  17. Radiation therapy of acromegaly.

    Science.gov (United States)

    Eastman, R C; Gorden, P; Glatstein, E; Roth, J

    1992-09-01

    Conventional megavoltage irradiation of GH-secreting tumors has predictable effects on tumor mass, GH, and pituitary function. 1. Further growth of the tumor is prevented in more than 99% of patients, with only a fraction of a percent of patients requiring subsequent surgery for tumor mass effects. 2. GH falls predictably with time. By 2 years GH falls by about 50% from the baseline level, and by 5 years by about 75% from the baseline level. The initial GH elevation and the size and erosive features of the sella turcica do not affect the percent decrease in GH from the baseline elevation. 3. With prolonged follow-up, further decrease in GH is seen at 10 and 15 years, with the fraction of surviving patients achieving GH levels less than 5 ng/mL approaching 90% after 15 years in our experience. Gender, previous surgery, and hyperprolactinemia do not seem to affect the response to treatment. Patients with initial GH greater than 100 ng/mL are significantly less likely to achieve GH values less than 5 ng/mL during long-term follow-up. 4. Hypopituitarism is a predictable outcome of treatment, is delayed, and may be more likely in patients who have had surgery prior to irradiation. There is no evidence that this complication is more common in patients with acromegaly than in patients with other pituitary adenomas receiving similar treatment. 5. Vision loss due to megavoltage irradiation--using modern techniques and limiting the total dose to 4680 rad given in 25 fractions over 35 days, with individual fractions not exceeding 180 rad--is extremely rare. The reported cases have occurred almost entirely in patients who have received larger doses or higher fractional doses. The theory that patients with acromegaly are prone to radiation-induced injury to the CNS and optic nerves and chiasm because of small vessel disease is not supported by a review of the reported cases. 6. Brain necrosis and secondary neoplasms induced by irradiation are extremely rare. 7. Although

  18. High incidence of thyroid cancer among patients with acromegaly.

    Science.gov (United States)

    Kaldrymidis, Dimitrios; Papadakis, Georgios; Tsakonas, Georgios; Kaldrymidis, Philippos; Flaskas, Theofanis; Seretis, Andreas; Pantazi, Eleni; Kostoglou-Athanassiou, Ifigenia; Peppa, Melpomeni; Roussou, Paraskevi; Diamanti-Kandarakis, Evanthia

    2016-01-01

    Several studies have suggested that patients with acromegaly have an increased risk of thyroid, colorectal, breast and prostate cancers. In this study we determined the prevalence of malignant neoplasms in patients with acromegaly. Cancer risk was evaluated in a cohort of 110 patients (M/F 48/62, age 58.63±13.8 years, range 30-86) with acromegaly. Mean age at diagnosis of acromegaly was 46.37±13.11 years. Mean period of time since diagnosis of acromegaly was 12.26+9.6 years. From 110 patients, cancer was diagnosed in 26 (23.6%) patients. Thyroid cancer was the most common cancer and was diagnosed in 13 patients (11.8%); other cancers encountered were gastric cancer (N=2), endometrial cancer (N-2), and breast cancer, colon cancer, prostate cancer (N-2), myelodysplastic syndrome, renal cell carcinoma, lung cancer and pancreatic carcinoma, one case each. Age, gender, age at the time of diagnosis of acromegaly, tumor size of pituitary adenoma and duration of disease were not associated with cancer development. This study suggests that patients with acromegaly have an increased risk of thyroid cancer and therefore they should undergo regular screening with hormonal and ultrasound evaluation of the thyroid and FNAB when required.

  19. Diagnosis and treatment of Cushing's disease without remission after transsphenoidal surgery%库欣病经蝶术后无效的诊疗分析

    Institute of Scientific and Technical Information of China (English)

    陈文轩; 张辉; 林毅; 李黎明

    2015-01-01

    Objective To discuss the diagnosis and treatment of Cushing's disease without remission after transsphenoidal surgery.Methods 12 patients of Cushing's disease without remission after transsphenoidal surgery were investigated in this retrospective study.There were 2 males and 10 females with an average age of 36 (21-46) years old.Cushing's disease was diagnosed with the typical symptoms and signs of Cushing's syndrome,endocrinological examination and CT or MRI.Transsphenoidal surgery was performed in all patients.None of the patients achieved remission after operation.Serum and urinary free cortisol (UFC)were still elevated 3 months after operation.Subtotal adrenalectomy combined with pituitary radiotherapy were performed in 5 patients,single subtotal adrenalectomy in 4,pituitary radiotherapy in 2 and bilateral adrenalectomy with adrenal autotransplantation in 1 patient.Results Those receiving subtotal adrenalectomy combined with pituitary radiotherapy had no recurrence or Nelson's syndrome during 20 years of follow-up,but 4 patients receiving single subtotal adrenalectomy recurred after 2 to 9 years after operation.2 patients achieved remission with pituitary radiotherapy 1 to 2 years later without recurrence during 5 years of follow-up.One patient who underwent bilateral adrenalectomy with adrenal autotransplantation needed steroid replacement and Nelson's syndrome occurred during 2 years of follow-up because transplanted adrenal gland had no function.Conclusion The diagnosis of Cushing's disease should be reconfirmed for patients without remission after transsphenoidal surgery and the treatment should be individualized based on the results of operation,pathology and imaging.%目的 探讨库欣病经蝶术后无效的诊断和治疗.方法 男2例,女10例,平均年龄36(21 ~46)岁.据典型库欣综合征表现、内分泌学和影像学检查诊断为库欣病而行经蝶手术.所有患者术后症状和体征均未缓解,术后3个月复查血

  20. PLASMA-LEVEL MONITORING OF MITOTANE (O,P'-DDD) AND ITS METABOLITE (O,P'-DDE) DURING LONG-TERM TREATMENT OF CUSHINGS-DISEASE WITH LOW-DOSES

    NARCIS (Netherlands)

    BENECKE, R; KELLER, E; VETTER, B; DEZEEUW, RA

    1991-01-01

    Mitotane (o,p'-DDD) can be used for the treatment of various adrenocortical diseases such as Cushing's syndrome, but the usual doses of 6-8 g per day are often associated with severe adverse effects. This paper reports the results of much lower doses of o.p'-DDD (0.5-2 g per day) in two patients wit

  1. Acromegaly Presenting as Cardiac Failure - A Case Report

    Directory of Open Access Journals (Sweden)

    Shohael Mahmud Arafat

    2011-09-01

    Full Text Available Acromegaly is characterized by chronic hypersecretion of growth hormone (GH and is associated with increased mortality rate because of the potential complications such as cardiovascular disease, respiratory disease, or malignancy, which are probably caused by the long-term exposure of tissues to excess GH, for at least 10 years, before diagnosis and treatment. Here we are reporting a case of acromegaly who initially presented with features of left ventricular failure for which she got herself admitted in CCU and was treated conservatively. Later on, after clinical examination and investigations she was diagnosed as a case of mitral regurgitation due to cardiomyopathy caused by acromegaly. After the successful transsphenoidal resection of the pituitary microadenoma, the level of GH was normalized and heart failure improved. Key words: acromegaly; heart failure; Pituitary microadenoma. DOI: http://dx.doi.org/10.3329/bsmmuj.v4i2.8644 BSMMU J 2011; 4(2:122-124

  2. An update on the treatment of acromegaly

    Directory of Open Access Journals (Sweden)

    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

  3. Current therapies and mortality in acromegaly.

    Science.gov (United States)

    Găloiu, S; Poiană, C

    2015-01-01

    Acromegaly is a rare disease most frequently due to a GH secreting pituitary adenoma. Without an appropriate therapy, life of patients with acromegaly can be shortened with ten years. Pituitary surgery is usually the first line therapy for GH secreting pituitary adenomas. A meta-analysis proved that mortality is much lower in operated patients, even uncured, than the entire group of patients and is similar with the general population in patients with GH30% utilization of SRAs reported a lower mortality ratio than studies with lower percentages of SRA administration. Although therapy with DA has long been used in patients with acromegaly, there are no studies reporting its effect on mortality, but its efficacy is limited by the low remission rate obtained. The use of conventional external radiotherapy, although with good remission rate in time, was linked with increased mortality, mostly due to cerebrovascular diseases. Mortality in acromegaly can be reduced to expected levels from general population by using modern therapies either in monotherapy or by using multimodal approaches in experienced centers.

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

    Directory of Open Access Journals (Sweden)

    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

  5. Cushing's ulcer: Further reflections

    OpenAIRE

    Kemp, William J.; Bashir, Asif; Dababneh, Haitham; Cohen-Gadol, Aaron A.

    2015-01-01

    Background: Brain tumors, traumatic head injury, and other intracranial processes including infections, can cause increased intracranial pressure and lead to overstimulation of the vagus nerve. As a result, increased secretion of gastric acid may occur which leads to gastro-duodenal ulcer formation known as Cushing's ulcer. Methods: A review of original records of Dr. Harvey Cushing's patients suffering from gastro-duodenal ulcers was performed followed by a discussion of the available litera...

  6. Acromegaly associated with gangliocytoma.

    LENUS (Irish Health Repository)

    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.

  7. Acromegaly without acral changes: A rare presentation

    Directory of Open Access Journals (Sweden)

    Nilanjan Sengupta

    2012-01-01

    Full Text Available Acromegaly is diagnosed clinically by the universal presence of acral enlargement and typical coarse facies. We report a short, elderly female developing acromegalic facies for last 10 years without acral overgrowth. She is a patient of primary hypothyroidism, well controlled for last 20 years. Acromegaly was proven by high level of serum insulin like growth factor-1 (IGF-1 and elevated and nonsuppressed level of growth hormone (GH, with other hormonal profile being undisturbed. She had mild insulin resistance and systemic hypertension in absence of any visual field defect. Magnetic resonance imaging (MRI of brain revealed pituitary hyperplasia without any detectable adenoma. No source of ectopic secretion of GH or growth hormone releasing hormone (GHRH could be localized. Therefore, atypical presentation of acromegaly needs high degree of suspicion even if some of the common features are lacking. Here, we have biochemically proved acromegaly with typical facies, short stature but no acral overgrowth and pituitary adenoma despite longstanding disease activity, and thus eluding diagnosis for years.

  8. [Clinical case of the month. Cardiac complications of acromegaly: a rare cause of dilated cardiomyopathy].

    Science.gov (United States)

    Devoitille, A; Beckers, A; Piérard, L A

    2012-04-01

    Acromegaly is a disease characterized by chronic growth hormone hypersecretion. Cardiovascular complications represent the main cause of death. We present here a rare case of dilated cardiomyopathy whose diagnosis revealed an acromegaly. This will provide the opportunity to review an uncommon disease and its recently reassessed prevalence.

  9. Characteristics of Acromegaly in Korea with a Literature Review

    Directory of Open Access Journals (Sweden)

    Jae Won Hong

    2013-09-01

    Full Text Available Acromegaly is a slowly progressive disease caused by excessive growth hormone (GH, which is related to a GH secreting pituitary tumor in most cases. Herein, we describe the epidemiology, clinical characteristics, and treatment of acromegaly in Korea with a literature review. The average annual incidence of acromegaly in Korea was 3.9 cases per million people, which was within the range of previous Western studies. The primary treatment for acromegaly was also transsphenoidal adenomectomy, which accounted for 90.4% of patients whose primary therapeutic options were known. The overall surgical remission rates were 89%, 87%, 64%, 70%, and 50% for modified Hardy classification I, II, IIIA, IIIB, and IV, respectively. An updated and larger study regarding the treatment outcome of medical/radiotherapy in Korean acromegalic patients is needed.

  10. Update on quality of life in patients with acromegaly.

    Science.gov (United States)

    Crespo, Iris; Valassi, Elena; Webb, Susan M

    2017-02-01

    In recent years, health-related quality of life (QoL) has been considered an important outcome for clinical management of acromegaly. Poor QoL has been described in acromegalic patients with active disease as well as after endocrine cure. It is known that acromegaly determines many physical problems and psychological dysfunctions that unavoidably impact on patients' QoL. Moreover, there is evidence that factors, such as radiotherapy or post-treatment GH deficiency also impair QoL in patients diagnosed with acromegaly. Thus, including the assessment of QoL in daily clinical practice has become fundamental to understand the consequences of acromegaly and the impact on the patients' daily life.

  11. The strange case of a patient affected by acromegaly with osteoporomalacia without hypogonadism

    Directory of Open Access Journals (Sweden)

    S. Caprio

    2011-09-01

    Full Text Available Acromegaly is a rare disease that, in the majority of cases, is due to the presence of a benign growth hormone (GH- producing tumor of the pituitary. Growth hormone has profound effects on linear bone growth, bone metabolism, and bone mass. In acromegaly, the skeletal effects of chronic GH excess have been mainly addressed by evaluating bone mineral density (BMD. Most data were obtained in patients with active acromegaly, and apparently high or normal BMD was observed in the absence of hypogonadism. The Autors describe a case of patient affected by acromegaly without hypogonadism with serious osteoporosis and biological signs of osteomalacia.

  12. Mortality in patients with pituitary disease.

    LENUS (Irish Health Repository)

    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. Microstructural brain changes in acromegaly: quantitative analysis by diffusion tensor imaging

    Science.gov (United States)

    Ilhan, M M; Alkan, A; Aralasmak, A; Akkoyunlu, M E; Kart, L; Tasan, E

    2014-01-01

    Objective: We examined brain diffusion changes of patients with acromegaly. We searched whether there are differences in apparent diffusion coefficient (ADC) and fractional anisotropy (FA) values between remission and non-remission patients with acromegaly and investigated any effect of time of hormone exposure on diffusion metrics. Methods: The values of FA and ADC were calculated in a total of 35 patients with acromegaly and 28 control subjects. Patients were subdivided into remission and non-remission groups. We looked at brain FA and ADC differences among the groups and looked for any relation between the diffusion changes and time of hormone exposure among the patients with acromegaly. Results: We found decreased FA and increased ADC values in some of the growth hormone responsive areas. There were no significant brain diffusion changes between remission and non-remission groups. The most affected areas were the hypothalamus, parietal white matter and pre-motor cortex in patients with acromegaly. In terms of hormone exposure time among the patients with acromegaly, there was no effect of disease duration on brain microstructural changes. Conclusion: All patients with acromegaly showed increased brain diffusion with no relation to disease duration and treatment status. We suggested that in patients with acromegaly, brain damage had already occurred in the subclinical period before symptom onset. Advances in knowledge: This study contributes to the understanding of the mechanisms in acromegaly. PMID:24734977

  14. 经鼻蝶手术治疗垂体磁共振扫描阴性的Cushing病%Transsphenoid surgery for Cushing disease with negative MRI finding

    Institute of Scientific and Technical Information of China (English)

    卞留贯; 孙青芳; 沈建康; 赵卫国; 宁光; 苏颋伟; 高广忠

    2009-01-01

    Objective To explore the diagnosis and treatment principle of Cushing disease with negative MRI.Methods Sixteen Cushing diseases with negative MRI were retrospectively reviewed.All the patients were diagnosed with Cushing disease based on clinical findings and laboratory criteria including loss of diurnal variation in serum cortisol levels,and/or elevated 24-h urinary free cortisol concentrations,normal or elevated ACTH levels.Five patients with failure of cortisol suppression after high-dose dexamethasone test,bilateral inferior petrosal sinus sampling (BIPSS) was performed.All patients underwent endonasal transsphenoid surgery.Adenoma was intraoperatively demonstrated in eleven cases,after a wide sellar dural opening.For four patients,the incision was made in the paramedian adenohypophysis on the side of the high ACTH gradient and adenomatous tissue was encountered with tenacious and yellow tissue.However,the partial hemihypophysectomy was performed in one patient,on the side with the higher ACTH gradient from BIPSS,because the BIPSS can not suggest the localization of the tumor.Results The early remission rate was achieved in ten cases (63%,10/16).The pathological finding showed the ACTH secreting adenoma in twelve cases,and pituitary corticotroph hyperplasia in 4 cases.Conclusion Transsphenoid surgery may be the choice for treating Cushing disease with negative MRI finding.%目的 探讨垂体MRI扫描未见异常的Cushing病的诊断和处理原则.方法 回顾性分析16例垂体MRI扫描未见异常的Cushing病患者,表现为血尿皮质醇升高、血皮质醇昼夜节律消失,正常或轻度升高的ACFH;其中大剂量地塞米松抑制试验中被抑制的11例,余5例通过舣侧岩下窦采血(BIPSS)证实垂体性Cushing病;手术采用经单鼻腔蝶窦入路,硬膜切开后发现肿瘤的11例,4例根据BIPSS提示的偏侧,ACTH高的一侧旁正中切开垂体,均发现肿瘤,肿瘤质地较韧,色泽偏黄.1例BIPSS不能提示肿瘤位置,

  15. Hormonal secretion and quality of life in Nelson syndrome and Cushing disease after long acting repeatable octreotide: a short series and update.

    Science.gov (United States)

    Arregger, Alejandro L; Cardoso, Estela M L; Sandoval, Olga B; Monardes Tumilasci, Elida G; Sanchez, Rocío; Contreras, Liliana N

    2014-01-01

    Clinical management of persistent adrenocorticotropin hormone (ACTH) excess in Nelson syndrome (NS) and Cushing disease (CD) remains a challenge. Somatostatin and its analogs as octreotide decrease ACTH secretion through somatostatin receptors of pituitary cells. To our knowledge, there are no reports on the effect of long-acting repeatable octreotide (oct-lar) on hormonal secretion and quality of life in patients with NS and CD who failed conventional therapy. Herein, we describe the effects of treatment with oct-lar (20 mg/month intramurally) in 1 woman with NS and 2 women with persistent CD. Oct-lar therapy reduced ACTH secretion and improved the quality of life in NS patient. By contrast, in CD patients, it failed to control ACTH and cortisol secretion, and the quality of life remained unchanged.

  16. Real-world comorbidities and treatment patterns of patients with acromegaly in two large US health plan databases

    OpenAIRE

    Burton, Tanya; Le Nestour, Elisabeth; Bancroft, Tim; Neary, Maureen

    2012-01-01

    Acromegaly is a rare, chronic, and debilitating disease that results from excessive growth hormone production. Clinically, this disease is associated with enlargement of soft tissue, excessive skeletal growth, and increased risk of cardiovascular disease. Acromegaly is often diagnosed late, when a wide range of comorbidities may already be present. First-line therapy for acromegaly is typically surgery; but a number of highly-specific pharmacological agents have recently enabled a more aggres...

  17. Ventricular tachycardia in acromegaly.

    Science.gov (United States)

    Arias, Miguel A; Pachón, Marta; Rodríguez-Padial, Luis

    2011-02-01

    Cases of sudden cardiac death have been reported in patients with acromegaly. Malignant ventricular arrhythmias may play an important role in this fatal complication, but the exact mechanisms are not well understood. We report on an acromegalic patient presenting with documented recurrent syncopal ventricular tachycardia.

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

    Science.gov (United States)

    Leporati, Paola; Fonte, Rodolfo; de Martinis, Luca; Zambelli, Alberto; Magri, Flavia; Pavesi, Lorenzo; Rotondi, Mario; Chiovato, Luca

    2015-05-12

    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.

  19. Symptoms and signs of acromegaly: an ongoing need to raise awareness among healthcare practitioners.

    Science.gov (United States)

    Zarool-Hassan, Redzuan; Conaglen, Helen M; Conaglen, John V; Elston, Marianne S

    2016-06-01

    INTRODUCTION Chronic excess growth hormone production results in acromegaly, a condition associated with widespread physical changes, including soft tissue and bony overgrowth. When untreated, acromegaly reduces life expectancy. Patients usually remain undiagnosed for years after the onset of symptoms, by which stage irreversible physical changes have often occurred. METHOD A cross-sectional questionnaire study involving patients with acromegaly from the Waikato Endocrine Unit and the New Zealand Acromegaly Society evaluated features of acromegaly that were present before diagnosis. The aim of this study was to identify acromegaly features that were most prevalent to promote increased awareness about the disease by healthcare providers. RESULTS 81 participants were included. The main pre-diagnosis physical changes participants reported were acral changes, alterations in facial features and oral symptoms. For some, these features were present for more than 10 years before the acromegaly diagnosis. Multiple co-morbidities associated with acromegaly were reported. Two-thirds of the participants felt that an earlier diagnosis was possible. Most participants were in contact with General Practitioners (GPs) and/or dentists before diagnosis. Endocrinologists had the highest diagnosis rate, followed by GPs. Dentists had a low diagnosis rate despite a high prevalence of oral symptoms among study participants. CONCLUSION Increased awareness of acromegaly among primary care clinicians is important as they are the first-point-of-contact with the healthcare system for most patients. Health professionals' early recognition of symptoms and signs of acromegaly would reduce delays in time-to-diagnosis, enable earlier treatment and may improve outcomes for patients with acromegaly. MESH KEYWORDS Acromegaly; symptoms; delayed diagnosis; clinicians; primary healthcare.

  20. Low frequency of cardniac arrhythmias and lack of structural heart disease in medically-naïve acromegaly patients: a prospective study at baseline and after 1 year of somatostatin analogs treatment.

    Science.gov (United States)

    Warszawski, Leila; Kasuki, Leandro; Sá, Rodrigo; Dos Santos Silva, Cintia Marques; Volschan, Isabela; Gottlieb, Ilan; Pedrosa, Roberto Coury; Gadelha, Mônica R

    2016-12-01

    The incidence of arrhythmias may be increased in acromegaly, but the pathophysiologic mechanisms involved are still unclear, and it has never been correlated with structural heart changes analyzed by the gold-standard method cardiac magnetic resonance (CMR). Evaluate the frequency of arrhythmias in drug-naïve acromegaly patients at baseline and after 1 year of somatostatin analogs (SA) treatment and to correlate the occurrence of arrhythmias with the presence of structural heart changes. Consecutive drug-naïve acromegaly patients were recruited. The occurrence of arrhythmias and structural heart changes were studied through 24-h Holter and CMR, respectively, at baseline and after 1-year SA treatment. Thirty-six patients were studied at baseline and 28 were re-evaluated after 1 year of SA treatment. There were 13 females and median age was 48 years (20-73 years). Nine patients (32 %) were controlled after treatment. No sustained arrhythmias were reported in the 24-h Holter. No arrhythmia-related symptoms were observed. Only two patients presented left ventricular hypertrophy and three patients presented fibrosis at baseline. There was no correlation of the left ventricular mass with the number of episodes of arrhythmias and they were not more prevalent in the patients presenting cardiac fibrosis. We found no sustained arrhythmias and a lack of arrhythmia-related symptoms at baseline and after 1 year of SA treatment in a contemporary cohort of acromegaly patients that also present a low frequency of structural heart changes, indicating that these patients may have a lower frequency of heart disease than previously reported.

  1. Radiation techniques for acromegaly

    Directory of Open Access Journals (Sweden)

    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.

  2. Acromegaly: Beyond surgery

    Directory of Open Access Journals (Sweden)

    Gaya Thanabalasingham

    2013-01-01

    Full Text Available Acromegaly is characterized by chronic, excess secretion of growth hormone (GH from a pituitary adenoma, and elevated hepatic insulin-like growth factor 1 (IGF-1 levels. Significant progress has been made in the development of medical therapies to achieve biochemical and symptomatic control in acromegaly. In this review we discuss the three currently available medical therapies, which include somatostatin analogs, dopamine agonists and pegvisomant. We describe a step-wise approach in which a somatostatin analog is followed by the addition of a dopamine agonist, and then if required the addition of or replacement by pegvisomant. New somatostatin agonists such as pasireotide, and the introduction of new orally-acting somatostatin agonists, should increase the therapeutic choices available in the near future.

  3. Acromegaly: Beyond surgery.

    Science.gov (United States)

    Thanabalasingham, Gaya; Grossman, Ashley B

    2013-07-01

    Acromegaly is characterized by chronic, excess secretion of growth hormone (GH) from a pituitary adenoma, and elevated hepatic insulin-like growth factor 1 (IGF-1) levels. Significant progress has been made in the development of medical therapies to achieve biochemical and symptomatic control in acromegaly. In this review we discuss the three currently available medical therapies, which include somatostatin analogs, dopamine agonists and pegvisomant. We describe a step-wise approach in which a somatostatin analog is followed by the addition of a dopamine agonist, and then if required the addition of or replacement by pegvisomant. New somatostatin agonists such as pasireotide, and the introduction of new orally-acting somatostatin agonists, should increase the therapeutic choices available in the near future.

  4. A Structural and Functional Acromegaly Classification

    Science.gov (United States)

    Cuevas-Ramos, Daniel; Carmichael, John D.; Cooper, Odelia; Bonert, Vivien S.; Gertych, Arkadiusz; Mamelak, Adam N.

    2015-01-01

    Context: GH-secreting pituitary adenomas exhibit heterogeneous natural history ranging from small tumors to large aggressive adenomas. Objective: To rigorously classify an acromegaly patient cohort defined by clinical, radiological, histopathological, and outcome characteristics. Design: Cross-sectional study. Setting: Tertiary referral pituitary center. Patients: Subjects were selected from a pituitary tumor research registry that includes 1178 patients with pituitary disease. Cluster analysis was performed on 338 acromegaly patients. Interventions: None. Main Outcome Measures: Biochemically active disease with elevated IGF-1 levels at follow-up. Results: Cluster analysis of all patients yielded 292 who were rigorously classified to three acromegaly types. Type 1 (50%) comprised older patients with the longest follow-up and most favorable outcomes, characterized by densely granulated, nonaggressive microadenomas and macroadenomas. Type 1 tumors extend to the sphenoid sinus more frequently than suprasellar extension (concave tumor image) and express abundant immunoreactive p21 and somatostatin receptor 2. Type 2 (19%) comprised noninvasive, densely or sparsely granulated macroadenomas, without significant extension (flat tumor image), with intermediate biochemical outcome. Type 3 (31%) was characterized by sparsely granulated aggressive macroadenomas and comprised patients with adverse therapeutic outcomes, despite receiving more treatments. These tumors extend to both the sphenoid sinus and suprasellar regions with commonly encountered optic chiasm compression (“peanut” magnetic resonance image), with low tumor p21 and somatostatin receptor 2 expression. Conclusions: After validation, this classification may be useful to accurately identify acromegaly patients with distinctive patterns of disease aggressiveness and outcome, as well as to provide an accurate tool for selection criteria in clinical studies. PMID:25250634

  5. Diabetic retinopathy in acromegaly

    Directory of Open Access Journals (Sweden)

    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.

  6. Acromegaly said to respond to proton therapy

    Energy Technology Data Exchange (ETDEWEB)

    Raymond, C.A.

    1988-02-12

    A news article is presented which discusses a new use for proton therapy. As physicians and physicists continue to refine the clinical applications for charged particles, they can point to at least one notable success story: the treatment of acromegaly, a disorder that afflicts an estimated 250 persons in the United States each year. Bernard Kliman, MD, reported at the annual Endocrine Society meeting in Indianapolis that his group at Harvard Medical School, Boston, and the Harvard cyclotron has cured 479 (85.5%) of 560 patients with acromegaly or gigantism. Cure is defined as reducing growth hormone level to less than 5 ..mu..g/L and shrinking the soft tissue growth characteristic of the disease.

  7. Early lung function abnormalities in acromegaly.

    Science.gov (United States)

    Benfante, A; Ciresi, A; Bellia, M; Cannizzaro, F; Bellia, V; Giordano, C; Scichilone, N

    2015-06-01

    Acromegaly is an insidious disorder caused by a pituitary growth hormone (GH)-secreting adenoma resulting in high circulating levels of GH and insulin-like growth factor I (IGF-I). Respiratory disorders are common complications in acromegaly, and can severely impact on quality of life, eventually affecting mortality. The present study aimed to explore structural and functional lung alterations of acromegalic subjects. We enrolled 10 consecutive patients (M/F: 5/5) affected by acromegaly. In all patients, magnetic resonance imaging (MRI) revealed the presence of pituitary tumor. All patients underwent clinical, lung functional, biological, and radiological assessments. Ten healthy age-matched subjects also served as controls. No statistically significant differences in lung function were detected between acromegalic and healthy subjects (p ≥ 0.05 for all analyses). However, the diffusing capacity for CO (TLCO) was significantly lower in the acromegalic group than in healthy subjects (TLCO% predicted: 78.1 ± 16 vs. 90 ± 6 %, respectively, p = 0.04; KCO% predicted: 77 ± 16 vs. 93 ± 5 %, p = 0.02, respectively). None of the lung function parameters correlated with duration of the disease, or with inflammatory marker of the airways. In acromegalics, biological (exhaled NO concentrations) and imaging (total lung volume, TLV, and mean lung density, MLD) evaluations were within normal values. The TLV measured by HRCT was 3540 ± 1555 ml in acromegalics, and the MLD was -711 ± 73 HU. None of the lung functional, radiological, and biological findings correlated with GH or IGF-I levels, and no correlation was found with duration of disease. In the current study, lung function evaluation allowed to detect early involvement of lung parenchyma, as assessed by TLCO and KCO, even in the absence of parenchymal density alterations of the lung by HRCT. These findings suggest to routinely include the carbon monoxide diffusing capacity in the lung function assessment for an

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

    Directory of Open Access Journals (Sweden)

    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

  9. Features at diagnosis of 324 patients with acromegaly did not change from 1981 to 2006; Acromegaly remains under-recognized and under-diagnosed

    Science.gov (United States)

    Reid, Tirissa J.; Post, Kalmon D.; Bruce, Jeffrey N.; Kanibir, M. Nabi; Reyes-Vidal, Carlos M.; Freda, Pamela U.

    2009-01-01

    BACKGROUND Traditionally, acromegaly evaded diagnosis until in its clinically obvious later stages when treatment is more difficult. Over the last 25 years diagnostic tests have improved, but whether clinical disease detection also improved was unknown so we tested if disease severity at diagnosis had changed from 1981 to 2006. METHODS Data on 324 consecutive acromegaly patients presenting from 1981–2006 at two New York City hospitals were collected by retrospective review (n=324) and by interview (n=200). The main complaint, acromegaly-associated co-morbidities, signs, symptoms, healthcare providers visited, pre-operative growth hormone (GH) and insulin-like growth factor I (IGF-I) levels and pituitary tumor size at diagnosis were compared in patients presenting in the earlier vs. later halves of the time period. RESULTS Times from symptom onset to diagnosis were 5.9 yr. (early) vs. 5.2 yr. (late)(p=ns). At diagnosis, 96% of early and late groups had facial feature changes and/or hand/foot enlargement. Co-morbidities included hypertension (HTN) 37 % (early) vs. 36% (late), carpal tunnel syndrome (24 vs. 24%), sleep apnea (13 vs. 29%)(p acromegaly patients were unchanged from 1981–2006. Most patients still have marked manifestations of acromegaly at diagnosis suggesting that acromegaly remains clinically under-recognized. Healthcare professionals should more commonly consider acromegaly, which can lead to earlier diagnosis and better treatment outcome. PMID:19473180

  10. A case of acromegaly complicated with diabetic ketoacidosis, pituitary apoplexy, and lymphoma.

    Science.gov (United States)

    Jiang, He-Jiun; Hung, Wei-Wen; Hsiao, Pi-Jung

    2013-12-01

    Acromegaly is always complicated with comorbidities and increased mortality. The disease activity and mortality outcomes are highly correlated to the level of growth hormone and insulin-like growth factor 1. A variety of clinical manifestations of acromegaly have been reported. We present a unique case where a 49-year-old male was diagnosed with acromegaly with a first manifestation as an episode of diabetic ketoacidosis. Because he refused any suggestion of treatment, a recurrent episode of diabetic ketoacidosis with pituitary apoplexy occurred. A huge B-cell lymphoma displaying as a huge facial mass followed within 1 year of the diagnosis of acromegaly. Death from advanced cancer ensued 3 years later. This clinical experience strongly reinforces the urgency of controlling growth hormone and insulin-like growth factor 1 as soon as possible once acromegaly is diagnosed.

  11. Changes in the management and comorbidities of acromegaly over three decades: the French Acromegaly Registry.

    Science.gov (United States)

    Maione, Luigi; Brue, Thierry; Beckers, Albert; Delemer, Brigitte; Petrossians, Patrick; Borson-Chazot, Françoise; Chabre, Olivier; François, Patrick; Bertherat, Jérôme; Cortet-Rudelli, Christine; Chanson, Philippe

    2017-05-01

    Acromegaly is a rare disease associated with chronic multisystem complications. National registries have been created in several countries. The French Registry contains data on acromegaly epidemiology, management and comorbidities recorded over more than three decades, retrospectively until 1999 and prospectively from 1999 to 2012. Data could be analyzed for 999 of the 1034 patients included in the registry (46% males). Disease control, defined as IGF-I normalization (adjusted for age and sex), was achieved in 75% of patients at the last follow-up visit. Half the patients with uncontrolled disease had IGF-I levels below 1.5 times the upper limit of normal (ULN). The proportion of patients with surgically cured disease did not change markedly over time, whereas the proportion of patients with uncontrolled disease fell and the proportion of patients with medically controlled disease rose. Cardiovascular, metabolic, respiratory and rheumatologic comorbidities and their outcomes were recorded for most patients, and no noteworthy overall deterioration was noted over time. Cancer occurred in 10% of patients, for a standardized incidence ratio of 1.34 (95% CI: 0.94-1.87) in men and 1.24 (0.77-1.73) in women. Forty-one patients died during follow-up, for a standardized mortality ratio of 1.05 (0.70-1.42). Most deaths were due to cancer. The majority of patients with acromegaly now have successful disease control thanks to the multistep management. The incidence of comorbidities following diagnosis of acromegaly is very low. Life expectancy is now close to that of the general population, probably owing to better management of the GH/IGF-I excess and comorbidities. © 2017 European Society of Endocrinology.

  12. Cardiac abnormalities in acromegaly. Pathophysiology and implications for management.

    Science.gov (United States)

    Vitale, Giovanni; Pivonello, Rosario; Lombardi, Gaetano; Colao, Annamaria

    2004-01-01

    Cardiovascular disease is claimed to be one of the most severe complications of acromegaly, contributing significantly to mortality in this disease. In fact, an excess of growth hormone (GH) and insulin-like growth factor 1 (IGF-I) causes a specific derangement of cardiomyocytes, leading to abnormalities in cardiac muscle structure and function, inducing a specific cardiomyopathy. In the early phase of acromegaly the excess of GH and IGF-I induces a hyperkinetic syndrome, characterized by increased heart rate and increased systolic output. Concentric hypertrophy is the most common feature of cardiac involvement in acromegaly, found in more than two thirds of patients at diagnosis. This abnormality is commonly associated with diastolic dysfunction and eventually with impaired systolic function ending in heart failure, if the GH/IGF-I excess is left untreated. In addition, abnormalities of cardiac rhythm and of heart valves have also been described in acromegaly. The coexistence of other complications, such as arterial hypertension and diabetes mellitus, aggravates acromegalic cardiomyopathy. Successful control of acromegaly induces a decrease in left ventricular mass and an improvement in diastolic function, while the effects of GH/IGF-I suppression on systolic function are more variable. However, since cardiovascular alterations in young patients with short disease duration are milder than in those with longer disease duration, it is likely to be easier to reverse and/or arrest acromegalic cardiomyopathy in young patients with early-onset disease. In conclusion, careful assessments of cardiac function, morphology, and activity are required in patients with acromegaly. An early diagnosis and prompt effective treatment are important in order to reverse acromegalic cardiomyopathy.

  13. Sleep apnea in active acromegaly.

    Science.gov (United States)

    Hart, T B; Radow, S K; Blackard, W G; Tucker, H S; Cooper, K R

    1985-05-01

    Previous case reports have shown an association between acromegaly and the sleep apnea syndrome (SAS). Some of the patients described had central SAS, raising the possibility that an elevation of the growth hormone (GH) level may cause a defect in respiratory drive. We determined the prevalence of SAS in 21 patients with a history of acromegaly. We separated them into two groups based on serum GH concentrations. Ten patients had active acromegaly (mean GH concentration, 62.2 ng/mL; range, 12.6 to 148 ng/mL), while 11 patients had inactive acromegaly (mean GH, 3.2 ng/mL; range, 0.7 to 6.4 ng/mL). Four of the ten patients with active acromegaly had SAS; none of the 11 patients with inactive acromegaly had SAS. Three patients with SAS had the purely obstructive type, and one had the mixed central and obstructive type. The hypercapnic ventilatory response was normal in all patients tested and was not influenced by the GH level. We conclude that SAS is associated with active acromegaly and that the GH level does not affect the hypercapnic ventilatory response. The absence of SAS in successfully treated patients suggests that it may resolve after a normal GH level is restored.

  14. Patient perspectives on the impact of acromegaly: results from individual and group interviews.

    Science.gov (United States)

    Gurel, Michelle H; Bruening, Paul R; Rhodes, Christine; Lomax, Kathleen G

    2014-01-09

    Acromegaly is a chronic condition resulting from a growth hormone-secreting pituitary tumor that can substantially impact patients' physical and emotional well-being. We sought to understand the impact of acromegaly on disease-related concerns and treatment choices from the patient perspective. The path to diagnosis, current disease management, interactions with the treating health care providers (HCPs), and support networks were also assessed. Acromegaly patients were recruited primarily from a patient support group (Acromegaly Community). In Phase I, ten patients participated over the course of 5 days in a moderated online discussion board and they answered questions about their disease. In Phase II, a separate nine-patient cohort participated in face-to-face interviews conducted during an acromegaly patient conference. Data were summarized qualitatively by grouping similar answers and quotations. Nineteen acromegaly patients were recruited across the two cohorts, and both groups shared similar concerns. They demonstrated a notable interest in understanding their disease and its treatment. Patients were focused on the impact of the disease on their life, and they expressed a desire to get beyond reminders of their disease. The patients described long journeys to a correct diagnosis and relief at having a name for their condition. Many shared a sense of shock at needing pituitary surgery and felt unsatisfied by the treatment decision process, motivating them to discuss it with other patients. Patients not connected to a patient support group reported feeling helpless and lonely. Most patients shared a desire to improve their general knowledge about acromegaly to spare others their protracted diagnostic period. Patients also reported hesitancy in asking questions or sharing details about the disease's impact on their lives with their HCPs. Acromegaly can be a life-changing diagnosis with profound, ongoing effects on patients' lives. Patients struggle with many

  15. ST-segment Elevation Acute Myocardial Infarction in a Patient with Acromegaly: A Case Report and Literature Review

    Directory of Open Access Journals (Sweden)

    Ming-Ying Lu

    2006-06-01

    Full Text Available Acromegaly is a disorder caused by the excess production of pituitary growth hormone and is characterized by the enlargement of the hands, feet and head. Increased morbidity and mortality with acromegaly is associated with cardiovascular complications, hypertension, glucose intolerance, cardiomyopathy and coronary artery disease. We report a case of acromegaly, which presented with ST-segment elevation acute myocardial infarction. The patient received successful primary transluminal coronary angioplasty with stent implantation. Acromegaly was suspected from typical appearance, and confirmed with hormonal examination and imaging of the pituitary mass. We discuss this case in comparison with previous literature.

  16. Recent developments in canine Cushing`s syndrome

    NARCIS (Netherlands)

    Galac, S.

    2010-01-01

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

  17. Recent developments in canine Cushing`s syndrome

    NARCIS (Netherlands)

    Galac, S.

    2010-01-01

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

  18. Recent developments in canine Cushing`s syndrome

    NARCIS (Netherlands)

    Galac, S.

    2010-01-01

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

  19. Recognizing the clinical manifestations of acromegaly: case studies.

    Science.gov (United States)

    Iuliano, Sherry L; Laws, Edward R

    2014-03-01

    To present case studies of a 36-year-old woman and a 73-year-old man who presented with the syndrome of acromegaly, and to provide primary care nurse practitioners (NPs) with the understanding of the clinical and laboratory features needed for early recognition and treatment of this fascinating disease. A comprehensive review of published literature on acromegaly is presented. The findings discussed include the history, physical examination, and diagnostic studies of two patients presenting in different ways. They both saw multiple healthcare providers, and had symptoms and signs eventually leading to the diagnosis of acromegaly. Acromegaly is the result of excessive amounts of growth hormone (GH) and insulin-like growth factor type 1 (IGF-1), almost always caused by a benign adenoma of the pituitary gland. This leads to coarse facial features, soft tissue swelling (including the tongue), enlargement of the hands and feet, respiratory problems, hypertension, diabetes mellitus, carpal tunnel syndrome, and osteoarthritis. Early recognition and appropriate referral can reverse some of the signs and symptoms over time, and lead to decreased mortality and a markedly improved quality of life. These cases exemplify the challenges faced by NPs and other healthcare providers in diagnosing patients with acromegaly. ©2013 The Author(s) ©2013 American Association of Nurse Practitioners.

  20. Case report: ACTH-secreting pituitary carcinoma metastatic to the liver in a patient with a history of atypical pituitary adenoma and Cushing's disease.

    Science.gov (United States)

    Joehlin-Price, Amy S; Hardesty, Douglas A; Arnold, Christina A; Kirschner, Lawrence S; Prevedello, Daniel M; Lehman, Norman L

    2017-04-18

    Pituitary carcinoma is a rare entity requiring the presence of metastasis to confirm its malignant potential. We report a case of pituitary carcinoma and discuss the diagnosis and management of this lesion in relation to the existing literature. The patient is a 51-year-old woman with Cushing's disease and intact adrenal glands who was diagnosed with metastatic pituitary carcinoma to the liver, 29 months after initial resection of an ACTH-secreting primary atypical pituitary adenoma (APA). Prior to detection of this metastasis the patient underwent repeat resection and radiotherapy for residual cavernous sinus disease. The metastatic lesion was detected by interval surveillance of serum ACTH and 24-hour urine cortisol, which despite stable pituitary MRI, were significantly elevated. These abnormalities prompted a PET scan that demonstrated hypermetabolic liver parenchyma, which was suspicious for metastasis on abdominal MRI. An ultrasound-guided liver biopsy demonstrated nests of moderately-differentiated cells with intermediate-sized, monotonous nuclei, distinct nucleoli, and abundant basophilic cytoplasm, confirmed by immunohistochemistry to represent metastatic pituitary carcinoma. The liver lesion was subsequently successfully removed by wedge resection. One year later, the patient's residual cavernous sinus disease grew markedly, and she was placed on dual-agent chemotherapy consisting of oral temozolomide and capecitabine, with stabilization of her intracranial disease to present, although liver metastases recurred. Pituitary carcinoma is a rare entity impossible to recognize as a primary tumor because its diagnosis by definition requires the presence of metastasis. Maintaining awareness of the entity and its precursor lesion APA is essential for its accurate pathologic diagnosis and appropriate management.

  1. Cushing's Disease Misdiagnosed as Primary Aldosteronism:A Case Report and Literature Review%库欣病误诊为原发性醛固酮增多症一例报告并文献复习

    Institute of Scientific and Technical Information of China (English)

    郭笑丹; 汪宁; 赵真真; 陈欢; 崔巍

    2015-01-01

    Objective To strengthen the understanding of atypical Cushing syndrome manifestations in Cushing dis-ease in order to improve the early diagnosis, reduce misdiagnosis or missed diagnosis and the mortality rates. Methods Clin-ical data of one patient with Cushing disease,who was admitted to our hospital in 2013 and misdiagnosed as having primary al-dosteronism, was retrospectively analyzed,and the related literature was reviewed. Results This patient with atypical Cushing syndrome manifestations, who suffered from hypertension and severe low blood potassium, was misdiagnosed as having primary aldosteronism. However the symptom did not improve after oral spironolactone and potassium. After inhibition test of small dose and large dose of dexamethasone, dynamic contrast-enhanced MRI appearances of pituitary, testing adrenocortical hor-mone(ACTH) by bilateral inferior petrosal sinus sampling ( BIPSS) in our hospital, this patient was diagnosed as having Cushing's disease. The levels of electrolyte, cortisol and ACTH returned to normal after left pituitary micro-adenoma enuclea-tion operation. Conclusion Clinicians should pay close attention to identifying atypical Cushing syndrome manifestations in Cushing disease patients who suffer from hypertension and severe low blood potassium and need to do relevant medical exami-nations in order to make early diagnosis and have early treatment, which will help to improve the prognosis of these patients.%目的:加强对无典型库欣综合征外貌的库欣病的认识,提高早期诊断水平,减少误漏诊,降低病死率。方法回顾分析我院2013年收治的1例误诊为原发性醛固酮增多症(原醛症)的库欣病的诊治经过及误诊原因,并复习相关文献。结果本例表现为高血压、严重低血钾,无典型库欣综合征外貌,曾在外院误诊为原醛症,予口服螺内酯、补钾等治疗效果差。在我院行小剂量及大剂量地塞米松抑制试验、垂体 MR +动

  2. Mandibular prognathism caused by acromegaly - a surgical orthodontic case.

    Science.gov (United States)

    Gosau, Martin; Vogel, Corinna; Moralis, Antonios; Proff, Peter; Kleinheinz, Johannes; Driemel, Oliver

    2009-01-01

    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.

  3. Restrictive extraocular myopathy: A presenting feature of acromegaly

    Directory of Open Access Journals (Sweden)

    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.

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

    Directory of Open Access Journals (Sweden)

    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.

  5. Macroglossia in acromegaly and hypothyroidism.

    Science.gov (United States)

    Wittmann, A L

    1977-04-29

    The tongues of two patients with macroglossia were examined at autopsy. One of the patients had acromegaly and the other had hypothyroidism. To evaluate the size of the enlarged tongues, the average weight of the tongue in the human adult was determined first in a series of 20 unselected autopsies, 10 males and 10 females (ages 44 to 85). The weight of the tongue was greater in males than in females and was directly correlated with the height of the subject. Cachexia had relatively little effect on the weight. In acromegaly (case 21) and myxedema (case 22) the tongue was enlarged by at least 50%. Histopathology showed enlargement of muscle fibers especially anteriorly in acromegaly and hypothyroidism, thickening of the epithelium and increased subepithelial and interstitial connective tissue. Incidental findings included venous thrombi and telangiectasia in the subepithelial connective tissue in both hypothyroidism and acromegaly and a corpus amylaceum and two islands of hyaline cartilage in the tongue of hypothyroidism.

  6. Diabetic ketoacidosis in a patient with acromegaly, a case report

    OpenAIRE

    O. Akha; R Rajabian; R. Aboutorabi; S. Teymouri

    2007-01-01

    Abstract Although impaired glucose tolerance affects 36% and overt diabetes mellitus can be seen in 30% of cases with acromegaly, diabetic ketoacidosis is rarely reported in patients with this disease. We present an unusual complication of acromegaly: a 22 year old woman with amenorrhea (6 month ago) and blurred vision of left eye (4 month ago) referred to Ghaem neurology clinic in Mashhad. With a growth hormone (GH) level of =100ng/ml, Insulin like growth factor-1 (IGF-1) =1560 ng/ml and pit...

  7. Review of current and emerging treatment options in acromegaly.

    Science.gov (United States)

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

    2015-10-01

    In almost every patient, acromegaly is caused by a growth hormone secreting pituitary adenoma. Clinical features are the result of excessive growth hormone secretion and the consecutive excess in insulin-like growth factor I levels. This results in somatic overgrowth and metabolic disturbances with a higher morbidity and mortality than in the general population. With optimal disease management, mortality can be reduced to that seen in the general population. The current treatment of acromegaly is based on a combination of surgery, radiotherapy and medical therapy. This review provides an overview of the current and upcoming therapies with a focus on medical therapy.

  8. QT dispersion in patients with acromegaly.

    Science.gov (United States)

    Unubol, Mustafa; Eryilmaz, Ufuk; Guney, Engin; Ture, Mevlut; Akgullu, Cagdas

    2013-04-01

    Acromegaly is a rare condition caused by a pituitary adenoma that secretes growth hormone. The mortality rate is 72 % higher in patients with acromegaly than in the general population according to meta-analyses. Mortality analysis has shown as many as 60 % of acromegalic patients die due to cardiovascular disease. Sudden cardiac death may occur in patients with acromegaly and malignant ventricular arrhythmia may play an important role in this fatal complication; however, the precise mechanism is not fully known. QT dispersion (dQT) is an electrophysiological factor known to be associated with a tendency for ventricular arrhythmia and sudden cardiac death. This study aimed to evaluate dQT as an early predictor of ventricular tachyarrhythmia, as sudden cardiac death commonly occurs in acromegalic patients. This cross-sectional case-control study enrolled 20 patients (10 female and 10 male) with acromegaly and 20 healthy controls (11 female and 9 male) after exclusion criteria were applied. Each participant underwent 12-lead electrocardiography, including ≥3 QRS complexes, at a speed of 25 mm/s after a 15-min rest. In each participant, the QT interval (beginning of the Q wave to the end of the T wave) was corrected (QTc) for heart rate using Bazett's formula [Formula: see text] QTc dispersion (dQTc) (QTc max - QTc min) was also calculated. There was no significant difference in median dQTc between the acromegalic patients (0.79 s) and the controls (0.45 s) (p > 0.05). Active acromegalic patients (n = 14) were estimated to have a median dQTc of 0.82 s, after excluding from the analysis six patients that were under full biochemical control, and that had randomly obtained growth hormone levels acromegaly might have an elevated risk for ventricular arrhythmia. We think that a non-invasive, simple and inexpensive marker-measurement of dQT-as part of cardiac monitoring could be valuable for screening complications in acromegalic patients.

  9. 60 YEARS OF NEUROENDOCRINOLOGY: Acromegaly.

    Science.gov (United States)

    Capatina, Cristina; Wass, John A H

    2015-08-01

    Acromegaly (ACM) is a chronic, progressive disorder caused by the persistent hypersecretion of GH, in the vast majority of cases secreted by a pituitary adenoma. The consequent increase in IGF1 (a GH-induced liver protein) is responsible for most clinical features and for the systemic complications associated with increased mortality. The clinical diagnosis, based on symptoms related to GH excess or the presence of a pituitary mass, is often delayed many years because of the slow progression of the disease. Initial testing relies on measuring the serum IGF1 concentration. The oral glucose tolerance test with concomitant GH measurement is the gold-standard diagnostic test. The therapeutic options for ACM are surgery, medical treatment, and radiotherapy (RT). The outcome of surgery is very good for microadenomas (80-90% cure rate), but at least half of the macroadenomas (most frequently encountered in ACM patients) are not cured surgically. Somatostatin analogs are mainly indicated after surgical failure. Currently their routine use as primary therapy is not recommended. Dopamine agonists are useful in a minority of cases. Pegvisomant is indicated for patients refractory to surgery and other medical treatments. RT is employed sparingly, in cases of persistent disease activity despite other treatments, due to its long-term side effects. With complex, combined treatment, at least three-quarters of the cases are controlled according to current criteria. With proper control of the disease, the specific complications are partially improved and the mortality rate is close to that of the background population.

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

    Science.gov (United States)

    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. Medical consequences of acromegaly: what are the effects of biochemical control?

    Science.gov (United States)

    Colao, Annamaria; Auriemma, Renata S; Pivonello, Rosario; Galdiero, Mariano; Lombardi, Gaetano

    2008-03-01

    This chapter discusses the effects of biochemical control of acromegaly on cardiovascular diseases, metabolic complications, respiratory abnormalities, malignancies and bone alterations. Acromegaly is associated with increased morbidity and mortality for cardiovascular and respiratory complications, whereas neoplasms seem to be a minor cause of increased risk of death. Other associated diseases are osteoarthritis, carpal tunnel syndrome, fatigue, visual abnormalities and reproductive disorders. Acromegaly results in premature death because of prolonged elevation of GH an IGF-I levels, and a strong biochemical control improves well-being and restores life expectancy to normal. The main goals of medical treatment of acromegaly include normalization of biochemical markers of disease activity, improvement in signs and symptoms of the disease, removal or reduction of tumor mass and preservation of pituitary function.

  12. Cardiovascular System Changes and Related Risk Factors in Acromegaly Patients: A Case-Control Study

    Directory of Open Access Journals (Sweden)

    Xiaopeng Guo

    2015-01-01

    Full Text Available Background. Cardiovascular complications are known to be the main determinants of reduced life expectancy and decreased quality of life in acromegaly patients. Our study aimed to provide insight into the cardiovascular changes that occur in acromegaly patients and to investigate the correlative risk factors. Methods. A total of 108 patients definitively diagnosed with acromegaly and 108 controls matched for age and gender were recruited into study and control groups, respectively. Standard echocardiography was performed on all of the participants, and data were collected and analyzed. Results. All acromegaly patients presented with structural cardiac changes, including a larger heart cavity, thicker myocardial walls, and increased great vessel diameters compared with the control group. Additionally, the acromegaly patients presented with reduced diastolic function. Aging and increased body mass index (BMI were correlated with myocardial hypertrophy and diastolic dysfunction; a longer disease duration was correlated with larger great vessel diameters. Conclusions. Ageing and increased BMI are independent risk factors for acromegalic cardiomyopathy, and a long disease duration results in the expansion of great vessels. Increased efforts should be made to diagnose acromegaly at an early stage and to advise acromegaly patients to maintain a healthy weight.

  13. Cardiovascular System Changes and Related Risk Factors in Acromegaly Patients: A Case-Control Study.

    Science.gov (United States)

    Guo, Xiaopeng; Gao, Lu; Zhang, Shuo; Li, Yilin; Wu, Yue; Fang, Ligang; Deng, Kan; Yao, Yong; Lian, Wei; Wang, Renzhi; Xing, Bing

    2015-01-01

    Background. Cardiovascular complications are known to be the main determinants of reduced life expectancy and decreased quality of life in acromegaly patients. Our study aimed to provide insight into the cardiovascular changes that occur in acromegaly patients and to investigate the correlative risk factors. Methods. A total of 108 patients definitively diagnosed with acromegaly and 108 controls matched for age and gender were recruited into study and control groups, respectively. Standard echocardiography was performed on all of the participants, and data were collected and analyzed. Results. All acromegaly patients presented with structural cardiac changes, including a larger heart cavity, thicker myocardial walls, and increased great vessel diameters compared with the control group. Additionally, the acromegaly patients presented with reduced diastolic function. Aging and increased body mass index (BMI) were correlated with myocardial hypertrophy and diastolic dysfunction; a longer disease duration was correlated with larger great vessel diameters. Conclusions. Ageing and increased BMI are independent risk factors for acromegalic cardiomyopathy, and a long disease duration results in the expansion of great vessels. Increased efforts should be made to diagnose acromegaly at an early stage and to advise acromegaly patients to maintain a healthy weight.

  14. Postoperative plasma cortisol levels predict long-term outcome in patients with Cushing's disease and determine which patients should be treated with pituitary irradiation after surgery

    Energy Technology Data Exchange (ETDEWEB)

    Imaki, Toshihiro; Tsushima, Toshio; Hizuka, Naomi; Odagiri, Emi; Murata, Yoji; Takano, Kazue [Tokyo Women' s Medical Univ. (Japan). Inst. of Clinical Endocrinology; Suda, Toshihiro

    2001-02-01

    Transsphenoidal surgery is the treatment of choice for ACTH-producing pituitary adenoma (Cushing's disease) and pituitary irradiation is widely considered the most appropriate treatment for patients with Cushing's disease for whom transsphenoidal surgery has been unsuccessful. We studied 49 consecutive patients who underwent transsphenoidal surgery for the treatment of Cushing's disease at Tokyo Women's Medical University from 1977-1997 with a mean follow-up duration of 87.6 months (range, 24-253 months). We examined the relationship between postoperative endocrinological data, assessed between 3 and 8 weeks after surgery, and long-term outcome and efficacy of pituitary irradiation after surgery. Long-term remission was defined as the regression of the symptom and signs of Cushing's syndrome, and restoration of normal levels of plasma ACTH, cortisol and urinary free cortisol, together with adequate suppression of morning plasma cortisol levels following the administration of low dose (1 mg) of dexamethasone. Thirty patients had no additional treatment after pituitary surgery. Only 1 of 25 patients (4%) whose postoperative plasma cortisol level was less than 2 {mu}g/dl developed recurrent disease whereas 3 out of 5 patients with postoperative plasma cortisol levels higher than 2 {mu}g/dl relapsed. Postoperative external pituitary radiation was used to treat the remaining 19 patients. Four patients who received radiation therapy had a low or undetectable postoperative plasma cortisol level (<2 {mu}g/dl, 56 nmol/L) and all of these patients developed hypopituitarism whereas 5 patients with subnormal plasma cortisol levels (2.0-10.0 {mu}g/dl) remained in remission. Among 10 patients with persistent disease after surgery, 6 entered remission 6-47 months after irradiation but one of them subsequently relapsed after 108 months. These results suggest that additional therapy should be avoided in patients with a postoperative plasma cortisol less

  15. Diabetic ketoacidosis in a patient with acromegaly

    NARCIS (Netherlands)

    Kopff, B; Mucha, S; Wolffenbuttel, B H; Drzewoski, J

    2001-01-01

    Abnormalities of glucose metabolism are a common feature of acromegaly. Overt diabetes mellitus develops in about 10-15% of patients. We present an unusual complication of acromegaly: a 37-year old man with a 2-year history of acromegaly developed diabetic ketoacidosis 3 weeks after transsphenoidal

  16. Diabetic ketoacidosis in a patient with acromegaly

    NARCIS (Netherlands)

    Kopff, B; Mucha, S; Wolffenbuttel, B H; Drzewoski, J

    2001-01-01

    Abnormalities of glucose metabolism are a common feature of acromegaly. Overt diabetes mellitus develops in about 10-15% of patients. We present an unusual complication of acromegaly: a 37-year old man with a 2-year history of acromegaly developed diabetic ketoacidosis 3 weeks after transsphenoidal

  17. [Cardiological findings in acromegaly].

    Science.gov (United States)

    Ferramosca, B; Bianchi, D; Serra, D; Savini, R; Villecco, A S; Bugiardini, R

    1987-12-31

    Acromegaly involves cardiovascular complications mostly due to the presence of hypertension, diabetes and atherosclerosis. However the appearance of cardiac decompensation and arrhythmias in the absence of predisposing factors tends to support the hypothesis of a specific myocardiopathy caused by excess GH. In order to assess the existence and course of subclinical cardiac alterations, 8 acromegaly patients were examined: 4 males and 4 females aged 31-56 with GH levels of 24-70 ng/ml (M + CD X 47 +/- 16) and no cardiovascular symptoms. One of the patients had moderate hypertension and 2 reduced glucose tolerance. The basal ECG showed sporadic ventricular extrasystoles in 2 cases and alterations compatible with left ventricular hypertrophy in another, while the effort ECG produced an asymptomatic depression of the ST segment in the hypertensive patient. The chest X-ray was normal in all cases. The echocardiography study investigated: the thickness of the interventricular septum (IVS = 13.9 +/- 2.8 mm), the thickness of the posterior wall of the left ventricle (LPW = 10.6 +/- 2.9 mm), the septum/posterior wall ratio (IVS/LPW = 1.3 +/- 0.2 the diastolic diameter (DD = 15.4 +/- 11.4 mm), the fraction of shortening (FS = 39.1 +/- 14.5%), the ejection fraction (EF = 64.1 +/- 18.4%) and revealed asymmetrical septal hypertrophy in 3 cases, concentric hypertrophy in another two. In two cases the DD and EF were distinctly altered. The patients were re-examined 2-4 years after surgical or radiation treatment. GH levels (M +/- SD = 10.3 +/- 10.1 ng/ml) were normal in 4 cases and still high, though lower in another two. The remaining two patients had borderline GH levels with high Sm-C. The ECG and chest X-ray were unchanged while echocardiography revealed a significant deterioration in heart function as far as DD (56.4 +/- 10.8 mm, p less than 0.05) were concerned with frankly pathological results in 4 and 3 cases respectively. These data confirm the view that most acromegalic

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

    Directory of Open Access Journals (Sweden)

    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.

  19. [Modern diagnosis and postoperative monitoring of acromegaly patients at a neurosurgical clinic].

    Science.gov (United States)

    Astaf'eva, L I; Kalinin, P L; Kadashev, B A

    Acromegaly is a severe disease associated with chronic overproduction of the growth hormone (GH) and insulin-like growth factor-1 (IGF-1), which is caused in most cases by pituitary adenoma. The main causes of mortality in acromegaly are cardiovascular diseases, respiratory diseases, and cancers. At present, the most effective treatment for acromegaly is surgical pituitary adenomectomy. Complete resection of pituitary tumors leads to the normalization of GH and IGF-1 levels, regression of symptoms, and a reduction in the risk of death. The article discusses the current criteria for diagnosis and remission of acromegaly after surgical adenomectomy as well as postoperative monitoring issues aligned with the recent guidelines of the Russian and international endocrinology associations.

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

    Science.gov (United States)

    Chabre, Olivier

    2014-04-01

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

  1. Neuromuscular complications of acromegaly.

    Science.gov (United States)

    Pickett, J B; Layzer, R B; Levin, S R; Scheider, V; Campbell, M J; Sumner, A J

    1975-07-01

    Seventeen consecutive acromegalic patients were evaluated for evidence of neuromuscular dysfunction and followed for 1 year after hypophysectomy. Before treatment, four patients had both a myopathy and the carpal tunnel syndrome, five had myopathy alone, four had carpal tunnel syndrome alone, and four had neither. The myopathy was caracterized by mild, strictly promixal weakness and flabbiness of muscles; electromyography revealed typical myopathic abnormalities, but serum enzymes and muscle biopsy usually were normal. The presence of myopathy or the carpal tunnel syndrrome could not be correlated with the magnitude of growth hormone elevation or any secondary endocrine derangement, but myopathy was associated with a longer duration of acromegaly. Carpal tunnel symptoms usually improved in the first 6 weeks after hypophysectomy, while myopathy improved more slowly and sometimes was detectable 1 year later.

  2. A case of acromegaly.

    Science.gov (United States)

    Siddiqui, N I; Chowdhury, K S; Rahman, S; Sarker, C B; Rahman, K M

    2003-01-01

    A fifty years old woman hailing from Purbadhala of Netrokona district complaining of gradual enlargement of hands, feet, nose and other acral parts of the body for about last eight years. She noticed coarsening of the skin and gradual protrusion of her lower jaw. She complained of headache, vertigo, frequent passage of urine, increased thirst, weight loss and fatiguability. She was found hypertensive having blood pressure 200/110 mm of Hg. Her appearance was coarse with rough skin. There were enlargement of hands, feet, nose, lower jaw with prognathism and enlargement of other acral parts. Investigations revealed high plasma glucose level, both fasting and 2 hrs. after glucose, high level of growth hormone, failure of suppression of growth hormone during OGTT. Thyroid function tests of the patient were found normal with increased heel pad size and enlarged sella turcica in all diameters. She was diagnosed as a case of acromegaly due to growth hormone hypersecretion.

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

    Science.gov (United States)

    Christofides, Elena A

    2016-01-01

    In acromegaly, achieving biochemical control (growth hormone [GH] level acromegaly is challenging because it is rooted in observing subtle clinical manifestations, and it is typical for acromegaly to evolve for up to 10 years before it is recognized. This results in chronic exposure to elevated levels of 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

  4. The role of an acute pasireotide suppression test in predicting response to treatment in patients with Cushing's disease: findings from a pilot study.

    Science.gov (United States)

    Trementino, L; Zilio, M; Marcelli, G; Michetti, G; Barbot, M; Ceccato, F; Boscaro, M; Scaroni, C; Arnaldi, G

    2015-09-01

    Pasireotide is a multireceptor-targeted somatostatin analog effective in the treatment of Cushing's disease (CD). We evaluate the value of an acute pasireotide suppression test (PST) in predicting response to medium/long-term treatment in CD. Nineteen patients with active CD were prospectively investigated at two referral centers from May 2013 to August 2014. Follow-up data (median 6 months; range 1-9 months) were available for sixteen patients. All patients received at 09:00 h a single subcutaneous (sc) injection of 600 μg pasireotide. Serum cortisol and plasma ACTH were assessed before, and every 2 h for 8 h after, drug administration. Late-night salivary cortisol (LNSC) was assessed before and after pasireotide administration. After acute PST, all patients were continued on pasireotide 600 μg sc twice a day. During PST, cortisol and ACTH levels quickly decreased in all patients except one with a mean percentage fall, respectively, of 48.9 ± 24.3 and 48.1 ± 25.4 % compared to baseline. LNSC decreased in about 82 % of patients (14/17) achieving a normalization in five of them. Pasireotide treatment was associated with a normalization of 24-h urinary-free cortisol at last follow-up in about 68 % of patients. A fall >27 % of LNSC during PST calculated by ROC curve was the best parameter in predicting a positive response to treatment with pasireotide (sensitivity 91 %; specificity 100 %; positive predictive value 100 %; negative predictive value 75 %). Acute PST may be useful to identify CD patients who will benefit from pasireotide treatment. A LNSC fall >27 % as well as a LNSC normalization during PST is associated with a probability of 100 % of achieving a favorable response to pasireotide treatment in the medium/long term.

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

    Energy Technology Data Exchange (ETDEWEB)

    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).

  6. Harvey Cushing's case series of trigeminal neuralgia at the Johns Hopkins Hospital: a surgeon's quest to advance the treatment of the 'suicide disease'.

    NARCIS (Netherlands)

    Adams, H.; Pendleton, C.; Latimer, K.; Cohen-Gadol, A.A.; Carson, B.S.; Quinones-Hinojosa, A.

    2011-01-01

    BACKGROUND: A review of Dr. Harvey Cushing's surgical cases at the Johns Hopkins Hospital provided insight into his early work on trigeminal neuralgia (TN). There was perhaps no other affliction that captured his attention in the way that TN did, and he built a remarkable legacy of successful treatm

  7. Diabetes in acromegaly, prevalence, risk factors, and evolution: data from the French Acromegaly Registry.

    Science.gov (United States)

    Fieffe, Sandrine; Morange, Isabelle; Petrossians, Patrick; Chanson, Philippe; Rohmer, Vincent; Cortet, Christine; Borson-Chazot, Françoise; Brue, Thierry; Delemer, Brigitte

    2011-06-01

    The French Acromegaly Registry records data of acromegalic patients' since 1992 in French, Belgian (Liège), and Swiss (Lausanne) centers. We studied the prevalence of diabetes in this population looking for risk factors. Patients from one of the centers (Reims) were then analyzed more thoroughly. This study has been conducted on all the patients recorded from 1999 until 2004 (519 patients). Evolution of cohorts' was reassessed in 2009. Of the different variables recorded in the registry: age, sex, body mass index (BMI), duration of acromegaly, GH, IGF1 and prolactin levels, pituitary tumor size, hormonal deficiencies, presence, duration and treatment of diabetes, hypertension, and rheumatological disease were analyzed. The prevalence of diabetes in the registry was 22.3%. Diabetic patients were older and had a higher BMI. Compared with the data of the French Social Security, acromegalic patients showed a more precocious apparition of diabetes and prevalence was higher in each age group. Compared with non-diabetic acromegalic subjects, diabetic patients had a more prolonged evolution of acromegaly before diagnosis. The levels of GH and IGF1 were not significantly different between the two groups. Only hypertension was significantly more frequent in diabetic patients. In our population, the prevalence of diabetes was estimated to be 22.3%. The GH and IGF1 levels did not appear as predictive factors for the presence of diabetes. On the contrary, age, BMI, and hypertension were significant risk factors as in the general population of type 2 diabetics.

  8. Treatment Factors That Influence Mortality in Acromegaly.

    Science.gov (United States)

    McCabe, John; Ayuk, John; Sherlock, Mark

    2016-01-01

    Acromegaly is a rare condition characterized by excessive secretion of growth hormone (GH), which is almost always due to a pituitary adenoma. Acromegaly is associated with significant morbidity such as hypertension, type 2 diabetes, cardiomyopathy, obstructive sleep apnoea, malignancy and musculoskeletal abnormalities. Acromegaly has also been associated with increased mortality in several retrospective studies. This review will focus on the epidemiological data relating to mortality rates in acromegaly, the relationship between acromegaly and malignancy, the role of GH and insulin-like growth factor-I in assessing the risk of future mortality, and the impact of radiotherapy and hypopituitarism on mortality.

  9. Familial acromegaly - case study of two sisters with acromegaly.

    Science.gov (United States)

    Malicka, Joanna; Świrska, Joanna; Nowakowski, Andrzej

    2011-01-01

    In the majority of cases, acromegaly is sporadic. However, it can also occur in a familial setting as a component of MEN-1, MEN-4, Carney complex (CNC) or as the extremely rare syndrome of isolated familial somatotropinoma (IFS), the latter belonging to familial isolated pituitary adenomas (FIPA). The diagnosis of IFS is based on the recognition of acromegaly/gigantism in at least two family members, given that the family is not affected by MEN-1, MEN-4 or CNC. The authors present a case study of two sisters: a 56 year-old patient (case no. 1) and a 61 year-old patient (case no. 2). In both sisters, acromegaly was recognised in the course of pituitary macroadenoma. Neither of the sisters showed features of MEN-1, MEN-4 or Carney complex. The authors suppose that the presented cases are manifestations of IFS. However, this diagnosis has not been confirmed yet because of the poor availability of genetic tests.

  10. Cushing syndrome after bilateral lensectomy.

    Science.gov (United States)

    Scherrer, Karin Sofia; Weitz, Marcus; Eisenack, Johannes; Truffer, Béatrice; Konrad, Daniel

    2015-03-01

    Iatrogenic Cushing syndrome induced by oral and parenteral corticosteroid administration is a well-known complication, and necessary precautions have to be taken. Cushing syndrome, however, following treatment with glucocorticoid-containing eye drops is a very rare complication. To the best of our knowledge, there have been only four reported cases in the literature. Herein, we present an infant boy who developed Cushing syndrome after receiving dexamethasone-containing eye drops after bilateral cataract extraction to prevent postoperative inflammatory complications. At the age of 5 months, after approx. 3 months of dexamethasone therapy, the patient presented with cushingoid facies, nephrocalcinosis and failure to grow. Iatrogenic Cushing syndrome was diagnosed and dexamethasone-containing eye drops were reduced and eventually stopped. Follow-up examinations revealed catch-up growth. Ocularly administered corticosteroids may have substantial systemic side effects in infants.

  11. Musculoskeletal complications of acromegaly: what radiologists should know about early manifestations.

    Science.gov (United States)

    Tagliafico, A; Resmini, E; Ferone, D; Martinoli, C

    2011-08-01

    The purpose of this article is to summarise the early musculoskeletal complications of acromegaly. Some of the early signs of acromegaly may be evaluated by the musculoskeletal radiologist. In the early stage of disease, peripheral nerve enlargement associated with carpal tunnel syndrome or cubital tunnel syndrome and thickening of retinacula, such as A1 pulley in trigger finger, represent the features that may be seen by radiologists and are worthy of an endocrinological evaluation. Due to the insidious nature of the disease, the diagnosis of acromegaly is significantly delayed. Few and nonspecific symptoms characterise the initial phases of the disease, and therefore, most patients will have generally consulted many specialists (most frequently musculoskeletal radiologists) before an adequate endocrinological assessment is performed. For this reason, initial clinical signs are much more important than symptoms for an early diagnosis of acromegaly. The first and most important therapeutic approach to acromegaly is early diagnosis, whereas the therapeutic goals are to eliminate morbidity and reduce mortality to the expected age- and sex-adjusted rates and prevent the development of systemic complications. Musculoskeletal radiologists should be aware that these features may be early manifestations of acromegaly. When both radiological and clinical abnormalities are present, an endocrinological workup is useful to diagnose the disease in an early phase.

  12. Challenges in the diagnosis and management of acromegaly: a focus on comorbidities

    OpenAIRE

    Abreu, Alin; Tovar, Alejandro Pinzón; Castellanos, Rafael; Valenzuela, Alex; Giraldo, Claudia Milena Gómez; Pinedo, Alejandro Castellanos; Guerrero, Doly Pantoja; Barrera, Carlos Alfonso Builes; Franco, Humberto Ignacio; Ribeiro-Oliveira, Antônio; VILAR, Lucio; Jallad, Raquel S.; Duarte, Felipe Gaia; Gadelha, Mônica; Boguszewski, Cesar Luiz

    2016-01-01

    Introduction Acromegaly is a rare, insidious disease resulting from the overproduction of growth hormone (GH) and insulin-like growth factor 1 (IGF-1), and is associated with a range of comorbidities. The extent of associated complications and mortality risk is related to length of exposure to the excess GH and IGF-1, thus early diagnosis and treatment is imperative. Unfortunately, acromegaly is often diagnosed late, when patients already have a wide range of comorbidities. The presence of co...

  13. Risk of malignant neoplasms in acromegaly: a case-control study.

    Science.gov (United States)

    Wolinski, K; Stangierski, A; Dyrda, K; Nowicka, K; Pelka, M; Iqbal, A; Car, A; Lazizi, M; Bednarek, N; Czarnywojtek, A; Gurgul, E; Ruchala, M

    2017-03-01

    Acromegaly is a chronic disease resulting from pathological oversecretion of growth hormone and subsequently insulin growth factor-1. Several complications of the disease have been reported, including cardiovascular diseases, respiratory disorders but also increased risk of benign and malignant neoplasms. The aim of the study was to evaluate the risk of malignant neoplasms in the patients with acromegaly in comparison with the control group. Medical documentation of acromegalic patients treated in one medical center between 2005 and 2016 has been analyzed. Results were compared with sex- and age-matched group of subjects with prolactinomas and hormonally inactive pituitary lesions hospitalized in the same department. Two hundred patients with acromegaly were included. Control group was composed of 145 patients. Any malignant neoplasm in anamnesis was present in 27 (13.5 %) patients with acromegaly and six (4.1 %) subjects from control group (p = 0.003). Thyroid cancer was present in 14 (7.0 %) patients with acromegaly and two (1.4 %) in control group (p = 0.02). Breast cancer was present in seven women (5.4 % of women) in acromegaly group but none of subjects in control group (p = 0.02). Colon cancer-4 (2.0 %) patients in acromegaly group and 0 in control group (p = 0.14). Malignant neoplasms are significantly more common in patients with acromegaly. Particularly, risk of thyroid cancer was increased over fivefold. Systematic screening for neoplastic diseases should be important part of follow-up in these patients. Further case-control studies are strongly indicated to evaluate which neoplasms are more common in acromegalic patients and what is the exact risk of malignancy.

  14. [Cushing's syndrome: review of a national caseload].

    Science.gov (United States)

    Contreras, P; Araya, V

    1995-03-01

    experienced 3 recurrences of the syndrome even after 8 years of successful removal of the pituitary adenoma. The remainder had to be cured by bilateral adrenalectomy. Seven out of 39 patients with Cushing's disease (18%) ultimately died for a variety of reasons; six out of 6 patients (100%) with adrenal carcinoma died of dissemination; two out of 2 adrenal adenomas cured and 1 out of the 3 ectopic ACTH syndromes died of dissemination of a malignant thymic carcinoma. We conclude that Cushing's syndrome is a serious, underdiagnosed disorder, which should be suspected and diagnosed by the non specialized physician and then referred to a specialized center for expert etiologic diagnosis and surgical therapy.

  15. Carpal tunnel syndrome and acromegaly.

    Science.gov (United States)

    Baum, H; Lüdecke, D K; Herrmann, H D

    1986-01-01

    50 patients with acromegaly and carpal tunnel syndrome have been examined electrophysiologically before and after transnasal operation of the pituitary adenoma. 32 of the 50 patients (64%) had symptoms of carpal tunnel syndrome. 13 of them had neurological deficits. 28 of the examined patients had pathological neurographical findings only. About 1 week post-operatively DL was decreased in 43%; in 10 out of 13 patients with neurological deficits DL decreased. GH was normalized in 80% and reduced to 5-10 micrograms/l in a further 10%. The investigation did not show whether the carpal tunnel syndrome only depended on a GH increase or on other factors also such as e.g., on the duration of symptoms or tissue changes. None of the patients had the transversal carpal ligament operated on. The coincidence between acromegaly and carpal tunnel syndrome was 64%. In 3 cases the carpal tunnel syndrome was the leading sign to the diagnosis of acromegaly.

  16. Cushing syndrome due to adrenal tumor

    Science.gov (United States)

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

  17. Patients with Acromegaly Presenting with Colon Cancer: A Case Series

    Directory of Open Access Journals (Sweden)

    Murray B. Gordon

    2016-01-01

    Full Text Available Introduction. Frequent colonoscopy screenings are critical for early diagnosis of colon cancer in patients with acromegaly. Case Presentations. We performed a retrospective analysis of the incidental diagnoses of colon cancer from the ACCESS trial (ClinicalTrials.gov identifier: NCT01995734. Colon cancer was identified in 2 patients (4.5%. Case  1 patient was a 36-year-old male with acromegaly who underwent transsphenoidal surgery to remove the pituitary adenoma. After surgery, the patient underwent routine colonoscopy screening, which revealed a 40 mm tubular adenoma in the descending colon. A T1N1a carcinoma was surgically removed, and 1 of 22 lymph nodes was positive for metastatic disease, leading to a diagnosis of stage 3 colon cancer. Case  2 patient was a 50-year-old male with acromegaly who underwent transsphenoidal surgery to remove a 2 cm pituitary adenoma. The patient reported severe cramping and lower abdominal pain, and an invasive 8.1 cm3 grade 2 adenocarcinoma with signet rings was identified in the ascending colon and removed. Of the 37 lymph nodes, 34 were positive for the presence of tumor cells, and stage 3c colon cancer was confirmed. Conclusion. Current guidelines for colonoscopy screening at the time of diagnosis of acromegaly and at appropriate follow-up intervals should be followed.

  18. Patients with Acromegaly Presenting with Colon Cancer: A Case Series

    Science.gov (United States)

    Nakhle, Samer; Ludlam, William H.

    2016-01-01

    Introduction. Frequent colonoscopy screenings are critical for early diagnosis of colon cancer in patients with acromegaly. Case Presentations. We performed a retrospective analysis of the incidental diagnoses of colon cancer from the ACCESS trial (ClinicalTrials.gov identifier: NCT01995734). Colon cancer was identified in 2 patients (4.5%). Case  1 patient was a 36-year-old male with acromegaly who underwent transsphenoidal surgery to remove the pituitary adenoma. After surgery, the patient underwent routine colonoscopy screening, which revealed a 40 mm tubular adenoma in the descending colon. A T1N1a carcinoma was surgically removed, and 1 of 22 lymph nodes was positive for metastatic disease, leading to a diagnosis of stage 3 colon cancer. Case  2 patient was a 50-year-old male with acromegaly who underwent transsphenoidal surgery to remove a 2 cm pituitary adenoma. The patient reported severe cramping and lower abdominal pain, and an invasive 8.1 cm3 grade 2 adenocarcinoma with signet rings was identified in the ascending colon and removed. Of the 37 lymph nodes, 34 were positive for the presence of tumor cells, and stage 3c colon cancer was confirmed. Conclusion. Current guidelines for colonoscopy screening at the time of diagnosis of acromegaly and at appropriate follow-up intervals should be followed. PMID:28025627

  19. Neurosurgical Treatment of Acromegaly.

    Science.gov (United States)

    Buchfelder, Michael; Feulner, Julian

    2016-01-01

    Surgical removal of as much tumor mass as possible is usually considered the first step of treatment in acromegaly, unless the patients are unfit for surgery or refuse an operation. To date, in almost all cases, minimally invasive, transsphenoidal microscopic or endoscopic approaches are used. Whether a curative approach is feasible or a debulking procedure is planned, can be anticipated on the basis of preoperative magnetic resonance imaging. It mostly depends on localization, size, and the invasive character of the lesion. The surgical results depend on tumor-related factors such as size, extension, the presence or absence of invasion, and the magnitude of IGF-1 and growth hormone oversecretion, respectively. However, even surgeon-related factors such as experience and case load of the centers have been shown to strongly affect surgical results and complication rates. A reoperation can be considered at various stages in the treatment algorithm. There are several new technical gadgets which might aid in the surgical procedure: navigation, the Doppler probe, and variants of intraoperative imaging.

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

    National Research Council Canada - National Science Library

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

    2015-01-01

    Cushing Syndrome is difficult to diagnose, and the comorbidities and persistent late effects of hypercortisolemia after treatment of the primary disease are challenging for the patient and the endocrinologist...

  1. Gender and magnetic resonance imaging classification-related differences in clinical and biochemical characteristics of Cushing's disease: a single-centre study

    Institute of Scientific and Technical Information of China (English)

    Chen Yufan; Mei Xiaobo; Jian Fangfang; Ma Qinyun; Chen Xiao; Bian Liuguan; Sun Qingfang

    2014-01-01

    Background Cushing's disease (CD) presents a remarkable preponderance in female gender,and a significant minority of patients with CD presented with negative magnetic resonance imaging (MRI) findings.The aim of this study was to evaluate gender-related and MRI classification-related differences in clinical and biochemical characteristics of CD.Methods We retrospectively studied 169 patients with CD,comprising 132 females and 37 males,and 33 patients had no visible adenoma on MRI.Results We observed that male and MRI-positive patients presented with high adrenocorticotropic hormone (ACTH) values (P <0.05).Female patients presented with higher prevalence of hirsutism and hyperpigmentation and lower prevalence of purple striae (P <0.05).The prevalence of buffalo-hump and hypertension was greater in MRI-negative patients (P <0.05).In addition,male patients with CD presented at a younger age compared with females (P <0.05).Patients with fatigue and hypokalaemia presented significantly higher urinary-free cortisol,ACTH and cortisol levels compared with patients without these symptoms (P <0.05).The prevalence of LH reduction,hyper total cholesterol (TC) and hyper low-density lipoprotein was more frequent in MRI-positive patients (P <0.05).Hyper-TC levels and PRL reduction were more frequent in males (P <0.05).T3,T4 and FT3 levels negatively correlated with age at diagnosis (r=-0.310,P <0.01; r=-0.191,P <0.05; r=-0.216,P <0.05).T3,T4,FT3 and FT4 levels significantly negatively correlated with 8-am plasma cortisol levels (r=-0.328,P <0.01; r=-0.195,P <0.05; r=-0.333,P <0.01; r=-0.180,P <0.05).Females presented higher total protein level (P <0.01) and lower blood urea nitrogen and serum creatinine levels (P <0.01),compared with male patients.Conclusions Carefulness and caution are required in all patients with CD,because of the complexity of clinical and biochemical characteristics in CD patients of different gender and MRI classification

  2. Prognosis of patients treated for Cushing syndrome.

    Science.gov (United States)

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

    2014-01-01

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

  3. Cushing's syndrome: why is diagnosis so difficult?

    Science.gov (United States)

    Aron, David C

    2010-06-01

    Practicing and perfecting the art of medicine demands recognition that uncertainty permeates all clinical decisions. When delivering clinical care, clinicians face a multiplicity of potential diagnoses, limitations in diagnostic capacity, and "sub-clinical" disease identified by tests rather than by clinical manifestations. In addition, clinicians must recognize the rapid changes in scientific knowledge needed to guide decisions. Cushing's syndrome is one of several disorders in which there may be considerable difficulty and delay in diagnosis. This article describes a current model of clinical reasoning, some of its challenges, and the application of the principles of clinical epidemiology to meet some of those challenges.

  4. Cyclic Cushing's syndrome: an overview.

    Science.gov (United States)

    Albiger, Nora Maria Elvira; Scaroni, Carla M; Mantero, Franco

    2007-11-01

    Cyclic Cushing's syndrome (CS) is a disorder in which glucocorticoid levels are alternately normal and high, the latter occurring in episodes that can last from a few days to several months. It is more common in children than in adults. Cyclic CS may be either of the two different forms of CS (ACTH-dependent or -independent CS). Clinically, it may present with one or many symptoms, depending on the duration of disease activity and the timing of the fluctuations. A serotoninergic influence, cyclic changes in central dopaminergic tone, spontaneous episodic hemorrhage in the tumor, and the action of inflammatory cytokines with antitumor properties are some of the mechanisms suggested to explain the physiopathology of this phenomenon but the exact mechanism remains to be clarified. The cyclic pattern of hypercortisolism can delay the final diagnosis of CS and make it difficult to interpret the results of dynamic tests. Patients may have paradoxical responses to dexamethasone that can reflect increasing or decreasing levels of endogenous activity. Hormone assessments have to be repeated periodically when a diagnosis of CS is suspected. The cyclic pattern can also interfere with medical treatment because patients may show unexpected clinical and biochemical signs of hypocortisolism when cortisol secretion cyclically returns to normal, so an accurate follow-up is mandatory in these patients.

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

    Science.gov (United States)

    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.

  6. Patient perspectives on the impact of acromegaly: results from individual and group interviews

    Directory of Open Access Journals (Sweden)

    Gurel MH

    2014-01-01

    Full Text Available Michelle H Gurel,1 Paul R Bruening,2 Christine Rhodes,2 Kathleen G Lomax31Neuroendocrine Clinical Center, Massachusetts General Hospital, Boston, MA, USA; 2Nicholas Research Associates International, New York, NY, USA; 3Medical Affairs, Ipsen Biopharmaceuticals, Inc., Basking Ridge, NJ, USAPurpose: Acromegaly is a chronic condition resulting from a growth hormone-secreting pituitary tumor that can substantially impact patients' physical and emotional well-being. We sought to understand the impact of acromegaly on disease-related concerns and treatment choices from the patient perspective. The path to diagnosis, current disease management, interactions with the treating health care providers (HCPs, and support networks were also assessed.Methods: Acromegaly patients were recruited primarily from a patient support group (Acromegaly Community. In Phase I, ten patients participated over the course of 5 days in a moderated online discussion board and they answered questions about their disease. In Phase II, a separate nine-patient cohort participated in face-to-face interviews conducted during an acromegaly patient conference. Data were summarized qualitatively by grouping similar answers and quotations.Results: Nineteen acromegaly patients were recruited across the two cohorts, and both groups shared similar concerns. They demonstrated a notable interest in understanding their disease and its treatment. Patients were focused on the impact of the disease on their life, and they expressed a desire to get beyond reminders of their disease. The patients described long journeys to a correct diagnosis and relief at having a name for their condition. Many shared a sense of shock at needing pituitary surgery and felt unsatisfied by the treatment decision process, motivating them to discuss it with other patients. Patients not connected to a patient support group reported feeling helpless and lonely. Most patients shared a desire to improve their general

  7. Genetics of gigantism and acromegaly.

    Science.gov (United States)

    Hannah-Shmouni, Fady; Trivellin, Giampaolo; Stratakis, Constantine A

    Gigantism and acromegaly are rare disorders that are caused by excessive GH secretion and/or high levels of its mediator, IGF-1. Gigantism occurs when excess GH or IGF-1 lead to increased linear growth, before the end of puberty and epiphyseal closure. The majority of cases arise from a benign GH-secreting pituitary adenoma, with an incidence of pituitary gigantism and acromegaly of approximately 8 and 11 per million person-years, respectively. Over the past two decades, our increasing understanding of the molecular and genetic etiologies of pituitary gigantism and acromegaly yielded several genetic causes, including multiple endocrine neoplasia type 1 and 4, McCune-Albright syndrome, Carney complex, familial isolated pituitary adenoma, pituitary adenoma association due to defects in familial succinate dehydrogenase genes, and the recently identified X-linked acrogigantism. The early diagnosis of these conditions helps guide early intervention, screening, and genetic counseling of patients and their family members. In this review, we provide a concise and up-to-date discussion on the genetics of gigantism and acromegaly. Published by Elsevier Ltd.

  8. Systemic complications of acromegaly: epidemiology, pathogenesis, and management.

    Science.gov (United States)

    Colao, Annamaria; Ferone, Diego; Marzullo, Paolo; Lombardi, Gaetano

    2004-02-01

    This review focuses on the systemic complications of acromegaly. Mortality in this disease is increased mostly because of cardiovascular and respiratory diseases, although currently neoplastic complications have been questioned as a relevant cause of increased risk of death. Biventricular hypertrophy, occurring independently of hypertension and metabolic complications, is the most frequent cardiac complication. Diastolic and systolic dysfunction develops along with disease duration; and other cardiac disorders, such as arrhythmias, valve disease, hypertension, atherosclerosis, and endothelial dysfunction, are also common in acromegaly. Control of acromegaly by surgery or pharmacotherapy, especially somatostatin analogs, improves cardiovascular morbidity. Respiratory disorders, sleep apnea, and ventilatory dysfunction are also important contributors in increasing mortality and are advantageously benefitted by controlling GH and IGF-I hypersecretion. An increased risk of colonic polyps, which more frequently recur in patients not controlled after treatment, has been reported by several independent investigations, although malignancies in other organs have also been described, but less convincingly than at the gastrointestinal level. Finally, the most important cause of morbidity and functional disability of the disease is arthropathy, which can be reversed at an initial stage, but not if the disease is left untreated for several years.

  9. New roles and challenges in neurosurgery of acromegaly.

    Science.gov (United States)

    Kleinberg, D L

    2005-01-01

    Acromegaly is a disease that shortens life expectancy (1-3) and causes severe systemic problems during life (4). It can arise and be recognized quickly if the onset is rapid, as in gigantism. Unfortunately there is usually a delay in diagnosis, on average 9 yr (4). The longer the delay the more likely patients are to develop partially or completely irreversible systemic problems, such as sleep apnea (5) and arthritis. Although some of the signs and co-morbidities of acromegaly are permanent, there is good evidence that cure of the disease reverses early mortality (3, 6, 7). It has been estimated that life expectancy is shortened by about 10 yr overall, and longer when diabetes or heart disease are already present at the time of diagnosis.

  10. Megaduodenum in a patient with acromegaly

    OpenAIRE

    Belinda George; Vinay, D; J Moolechery; Mathew, V; Anantharaman, R.; Ayyar, V; Bantwal, G

    2012-01-01

    Introduction: Acromegaly is associated with enlargement of all organs including the gastro intestinal system. However, there are no previous reports of occurrence of megaduodenum in patients with acromegaly. Discussion: We present the case of a 47 year old male who was diagnosed to have acromegaly 13 years ago and had undergone transsphenoidal surgery twice with incomplete removal of the pituitary macro-adenoma and received radiotherapy following the second surgery. Patient has been on replac...

  11. Clinical Manifestations and Diagnosis of Acromegaly

    OpenAIRE

    Gloria Lugo; Lara Pena; Fernando Cordido

    2012-01-01

    Acromegaly and gigantism are due to excess GH production, usually as a result of a pituitary adenoma. The incidence of acromegaly is 5 cases per million per year and the prevalence is 60 cases per million. Clinical manifestations in each patient depend on the levels of GH and IGF-I, age, tumor size, and the delay in diagnosis. Manifestations of acromegaly are varied and include acral and soft tissue overgrowth, joint pain, diabetes mellitus, hypertension, and heart and respiratory failure. Ac...

  12. Outcome of adrenalectomy for subclinical hypercortisolism and Cushing syndrome.

    Science.gov (United States)

    Raffaelli, Marco; De Crea, Carmela; D'Amato, Gerardo; Gallucci, Pierpaolo; Lombardi, Celestino P; Bellantone, Rocco

    2017-01-01

    We compared operative and metabolic outcomes in patients with subclinical Cushing syndrome and Cushing syndrome caused by unilateral adrenal lesion, aiming to clarify the role of glucocorticoid replacement treatment in patients with subclinical Cushing syndrome after adrenalectomy. The medical records of all the patients who underwent unilateral adrenalectomy for subclinical Cushing syndrome or Cushing syndrome were reviewed. Diagnostic criteria for subclinical Cushing syndrome were a pathologic dexamethasone suppression test plus 2 additional criteria. Twenty-nine patients with subclinical Cushing syndrome and 50 with Cushing syndrome were identified. No significant difference was found between patients with subclinical Cushing syndrome and Cushing syndrome regarding lesion size, operative time, and hospital stay. Two patients out of 29 with subclinical Cushing syndrome and 3 out of 50 patients with Cushing syndrome experienced Clavien-Dindo grade II complications (P = .87). All the patients required postoperative glucocorticoid replacement that was discontinued within 6 months in 28 of the 29 patients with subclinical Cushing syndrome and in 3 out of 50 Cushing syndrome patients (P Cushing syndrome and Cushing syndrome. Hypercortisolism was resolved in all the cases. Operative and metabolic outcomes of adrenalectomy are similar in subclinical Cushing syndrome and Cushing syndrome. Postoperative glucocorticoid replacement treatment is advisable in all patients with subclinical Cushing syndrome. Prolonged adrenal insufficiency is more frequent in Cushing syndrome patients. Copyright © 2016 Elsevier Inc. All rights reserved.

  13. Neurocognitive Function in Acromegaly after Surgical Resection of GH-Secreting Adenoma versus Naïve Acromegaly

    Science.gov (United States)

    Martín-Rodríguez, Juan Francisco; Madrazo-Atutxa, Ainara; Venegas-Moreno, Eva; Benito-López, Pedro; Gálvez, María Ángeles; Cano, David A.; Tinahones, Francisco J.; Torres-Vela, Elena; Soto-Moreno, Alfonso; Leal-Cerro, Alfonso

    2013-01-01

    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 better

  14. Peripheral nervous system assessment in acromegaly patients under somatostatin analogue therapy.

    Science.gov (United States)

    Alibas, H; Gogas Yavuz, D; Kahraman Koytak, P; Uygur, M; Tanridag, T; Uluc, K

    2017-01-01

    Acromegaly is known to affect peripheral nervous system (PNS) causing carpal tunnel syndrome (CTS) and polyneuropathy. The frequency of these disorders and the evaluation methods vary among studies. In the present study, we aimed to examine PNS of acromegaly patients under somatostatin analogue (SSA) therapy. Forty-eight acromegaly patients (26 F/22 M, 45.58 ± 11.6 years) under SSA treatment and 44 healthy controls (25 F/19 M, 47.46 ± 8.7 years) were assessed by symptom questionnaires, neurologic examination and electrophysiological studies. 87.5 % of the acromegaly patients had at least one abnormal finding regarding PNS. With the incorporation of palm-wrist median nerve conduction velocity method, we detected CTS in 50 % of patients. Polyneuropathy was less frequent (29.2 %). Both conditions were independent from the coexisting diabetes mellitus (p = 0.22 for CTS, p = 0.71 for polyneuropathy). Polyneuropathy but not CTS was more common among biochemically uncontrolled acromegaly patients rather than those under control (p = 0.03; p = 0.68, respectively). Our findings emphasize the high prevalence of peripheral nervous system involvement in acromegaly patients under SSA therapy and importance of neurological evaluation of these patients. Early diagnosis and treatment of the disease may reduce the PNS involvement.

  15. Treatments, complications, and healthcare utilization associated with acromegaly: a study in two large United States databases.

    Science.gov (United States)

    Broder, M S; Neary, M P; Chang, E; Cherepanov, D; Katznelson, L

    2014-08-01

    The economic burden of acromegaly in the US has been largely unknown. We describe the prevalence of treatment patterns, complication rates, and associated healthcare utilization and costs of acromegaly in the US. Patients were identified between 1/1/2002 and 12/31/2009 in claims databases. During 1-year after each continuously-enrolled patient's first acromegaly claim, pharmacy and medical claims were used to estimate outcomes. Regression models were used to adjust outcomes. There were 2,171 acromegaly patients (mean age: 45.3 years; 49.7% female); 77.8% received the majority of their care from non-endocrinologists. Pharmacologic treatment was used by 30.8% of patients: octreotide-LAR in 18.6%, dopamine agonists in 9.8%, short-acting octreotide in 4.7%, pegvisomant in 4.1%, and lanreotide in 1.2%; 56% had biochemical monitoring. Comorbidities were common, ranging from 6.6% (colon neoplasms) to 25.6% (musculoskeletal abnormalities). Mean healthcare costs were $24,900. Adjusted analyses indicated comorbidities increased the odds of hospitalization: by 76% for musculoskeletal abnormalities; 193% for cardiovascular abnormalities; and 56% for sleep apnea (p acromegaly patients. Cardiovascular complications nearly tripled the odds of hospitalization (OR 2.93) and increased annual mean cost by $13,331. Adequate management of this disease may be able to reduce health care utilization and cost associated with these complications and with acromegaly in general.

  16. E2F1-mediated human POMC expression in ectopic Cushing's syndrome.

    Science.gov (United States)

    Araki, Takako; Liu, Ning-Ai; Tone, Yukiko; Cuevas-Ramos, Daniel; Heltsley, Roy; Tone, Masahide; Melmed, Shlomo

    2016-11-01

    Cushing's syndrome is caused by excessive adrenocorticotropic hormone (ACTH) secretion derived from pituitary corticotroph tumors (Cushing disease) or from non-pituitary tumors (ectopic Cushing's syndrome). Hypercortisolemic features of ectopic Cushing's syndrome are severe, and no definitive treatment for paraneoplastic ACTH excess is available. We aimed to identify subcellular therapeutic targets by elucidating transcriptional regulation of the human ACTH precursor POMC (proopiomelanocortin) and ACTH production in non-pituitary tumor cells and in cell lines derived from patients with ectopic Cushing's syndrome. We show that ectopic hPOMC transcription proceeds independently of pituitary-specific Tpit/Pitx1 and demonstrate a novel E2F1-mediated transcriptional mechanism regulating hPOMC We identify an E2F1 cluster binding to the proximal hPOMC promoter region (-42 to +68), with DNA-binding activity determined by the phosphorylation at Ser-337. hPOMC mRNA expression in cancer cells was upregulated (up to 40-fold) by the co-expression of E2F1 and its heterodimer partner DP1. Direct and indirect inhibitors of E2F1 activity suppressed hPOMC gene expression and ACTH by modifying E2F1 DNA-binding activity in ectopic Cushing's cell lines and primary tumor cells, and also suppressed paraneoplastic ACTH and cortisol levels in xenografted mice. E2F1-mediated hPOMC transcription is a potential target for suppressing ACTH production in ectopic Cushing's syndrome. © 2016 Society for Endocrinology.

  17. Coagulopathy in Cushing's syndrome.

    Science.gov (United States)

    Trementino, Laura; Arnaldi, Giorgio; Appolloni, Gloria; Daidone, Viviana; Scaroni, Carla; Casonato, Alessandra; Boscaro, Marco

    2010-01-01

    A hypercoagulable state and its consequent increased incidence of thromboembolic complications are reported in patients with Cushing's syndrome (CS). These alterations are related to cortisol excess that induces prothrombotic changes in blood by several and complex mechanisms including increased levels of clotting factors, mainly factor VIII and von Willebrand factor (VWF) and impaired fibrinolytic capacity. However, it has recently been observed that the increase in VWF levels is not a constant feature of CS and that VWF response to glucocorticoids is genetically determined and depends on the presence of particular polymorphisms in the VWF gene promoter. The risk of venous thromboembolism is moreover enhanced in patients with CS by additional endogenous and exogenous risk factors such as obesity, bed rest, surgery and invasive diagnostic procedures like inferior petrosal sinus (IPS) sampling. In line with all these data, patients with active CS should be treated as having a prothrombotic disorder and undergo antithrombotic prophylaxis during IPS sampling. Special care should be taken in the immediate perioperative period in order to avoid thromboembolic events. In the absence of prospective randomized trials, preventive antithrombotic treatment (best with heparin) during IPS sampling and low-dose heparin treatment early after surgery should be suggested.

  18. Patients' experience of nursing with Acromegaly

    DEFF Research Database (Denmark)

    Jensen, Jonna Gintberg; Høi, Henriette Brahe

    for participation. An Interview guide was used, and interviews were conducted until data saturation was achieved. Interviews were recorded, and transcription was performed by the researcher. The patients’ statements were categorized by Eriksson's disease, care and life disorders and the three operational levels...... from Kvale’s analysis model were used. The research project's most spectacular findings were that: 1 ) The patients didn’t experience that caregivers had knowledge of the disease and symptoms 2 ) Other patients with Acromegaly were an important source of information 3) Being a teaching object...... 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...

  19. Acromegaly and gigantism in the medical literature. Case descriptions in the era before and the early years after the initial publication of Pierre Marie (1886).

    Science.gov (United States)

    de Herder, Wouter W

    2009-01-01

    In 1886 Pierre Marie used the term "acromegaly" for the first time and gave a full description of the characteristic clinical picture. However several others had already given clear clinical descriptions before him and sometimes had given the disease other names. After 1886, it gradually became clear that pituitary enlargement (caused by a pituitary adenoma) was the cause and not the consequence of acromegaly, as initially thought. Pituitary adenomas could be found in the great majority of cases. It also became clear that acromegaly and gigantism were the same disease but occurring at different stages of life and not different diseases as initially thought. At the end of the 19th and beginning of the 20th century most information was derived from case descriptions and post-mortem examinations of patients with acromegaly or (famous) patients with gigantism. The stage was set for further research into the pathogenesis, diagnosis and therapy of acromegaly and gigantism.

  20. Acromegaly at diagnosis in 3173 patients from the Liège Acromegaly Survey (LAS) Database

    Science.gov (United States)

    Petrossians, Patrick; Daly, Adrian F; Natchev, Emil; Maione, Luigi; Blijdorp, Karin; Sahnoun-Fathallah, Mona; Auriemma, Renata; Diallo, Alpha M; Hulting, Anna-Lena; Ferone, Diego; Hana, Vaclav; Filipponi, Silvia; Sievers, Caroline; Nogueira, Claudia; Fajardo-Montañana, Carmen; Carvalho, Davide; Hana, Vaclav; Stalla, Günter K; Jaffrain-Réa, Marie-Lise; Delemer, Brigitte; Colao, Annamaria; Brue, Thierry; Neggers, Sebastian J C M M; Zacharieva, Sabina; Chanson, Philippe

    2017-01-01

    Acromegaly is a rare disorder caused by chronic growth hormone (GH) hypersecretion. While diagnostic and therapeutic methods have advanced, little information exists on trends in acromegaly characteristics over time. The Liège Acromegaly Survey (LAS) Database, a relational database, is designed to assess the profile of acromegaly patients at diagnosis and during long-term follow-up at multiple treatment centers. The following results were obtained at diagnosis. The study population consisted of 3173 acromegaly patients from ten countries; 54.5% were female. Males were significantly younger at diagnosis than females (43.5 vs 46.4 years; P 3100 patients is the largest international acromegaly database and shows clinically relevant trends in the characteristics of acromegaly at diagnosis. PMID:28733467

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

    Science.gov (United States)

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

    2017-09-01

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

  2. Acromegaly Quality of Life Questionnaire (AcroQoL

    Directory of Open Access Journals (Sweden)

    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. [Anesthetic management of a patient with acromegaly complicated with hyperthyroidism].

    Science.gov (United States)

    Kawakubo, A; Ariyoshi, S; Fukui, S; Shimada, M; Haseba, S; Gotoh, Y

    1989-05-01

    A rare anesthetic experience of a 30-year-old woman with acromegaly complicated with Basedow's disease is reported. After the thyroid function was successfully controlled by drug therapy, resection of pituitary adenoma was performed under general anesthesia. Anesthesia was induced and maintained with NLA. No problem was observed during the operation and postoperative period. Careful attention should be paid to the management of circulation, respiration, metabolism and endocrinium through the perioperative period.

  4. Double benefit of long-acting somatostatin analogs in a patient with coexistence of acromegaly and ulcerative colitis.

    Science.gov (United States)

    Yarman, S; Yalın, G Y; Dogansen, S C; Canbaz, B; Tanrıkulu, S; Akyuz, F

    2016-10-01

    Somatostatin analogs control GH/IGF-1 excess in acromegaly. Somatostatin receptors also mediate the complex effects of somatostatin on the gastrointestinal tract and may be defensive in inflammatory bowel diseases, such as ulcerative colitis. We present a patient who showed good response to long-acting octreotide (OCT-LAR) treatment in terms of both acromegaly and ulcerative colitis (UC). A 58-year-old female patient with diagnosis of acromegaly and ulcerative colitis was started on long-acting somatostatin treatment as a first-line treatment for acromegaly as she refused to undergo transsphenoidal surgery. During the follow-up period, a significant amelioration was also observed in the course of ulcerative colitis, and clinical remission of both diseases was achieved uneventfully. Somatostatin appears to be a promising candidate in the treatment of inflammatory bowel diseases. © 2016 John Wiley & Sons Ltd.

  5. Pasireotide: a novel treatment for patients with acromegaly

    Directory of Open Access Journals (Sweden)

    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

  6. Patterns of pharmacologic treatment in US patients with acromegaly.

    Science.gov (United States)

    Broder, Michael S; Chang, Eunice; Ludlam, William H; Neary, Maureen P; Carmichael, John D

    2016-05-01

    To establish a baseline pattern of care across academic and community settings, it is important to examine the contemporary treatment of acromegaly. We characterized medical treatment patterns for acromegaly in the US to develop a basis for tracking concordance with guidelines. Acromegaly patients were identified in two commercial claims databases for this retrospective analysis. Study subjects had ≥2 medical claims with acromegaly (ICD-9-CM code 253.0) and ≥1 claim for pharmacotherapy (bromocriptine, cabergoline, octreotide SA, octreotide LAR, lanreotide, or pegvisomant) in the study timeframe (1 January 2002-31 December 2013). Patients were considered newly treated if they were continuously enrolled for ≥6 months before first observed treatment and had no claim for pharmacologic treatment during that time. Outcomes included various pharmacotherapies, including combination treatments, and differences between lines of therapy. A total of 3150 patients had ≥1 pharmacotherapy (mean age: 46.5 years; 50.1% were female); 1471 were newly treated. Somatostatin receptor ligands (SRLs) were the most common drug class used first line (57.2%); cabergoline (27.8%) was the most common treatment, followed by octreotide LAR (22.3%) and lanreotide (19.7%). SRLs were also the most commonly used second-line (42.8%) and third-line pharmacotherapies (43.9%), with combination therapy (23.2%) and octreotide LAR (19.8%) as the most commonly used treatments, respectively. This study, representing the largest claims-based analysis of acromegaly to date, used two databases across a 12 year period to examine complex treatment patterns in a difficult-to-study disease. Although wide variation in acromegaly treatment patterns exists in US clinical practice, in first-line, second-line, and third-line therapy, SRL was the most commonly used drug class. Drug combinations also varied considerably across lines of therapy. The switching between different monotherapies and varied use of drugs

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

    OpenAIRE

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

    1989-01-01

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

  8. High-resolution 18F-fluorodeoxyglucose positron emission tomography and magnetic resonance imaging for pituitary adenoma detection in Cushing disease

    Science.gov (United States)

    Chittiboina, Prashant; Montgomery, Blake K.; Millo, Corina; Herscovitch, Peter; Lonser, Russell R.

    2016-01-01

    OBJECT High-resolution PET (hrPET) performed using a high-resolution research tomograph is reported as having a resolution of 2 mm and could be used to detect corticotroph adenomas through uptake of 18F-fluorodeoxyglucose (18F-FDG). To determine the sensitivity of this imaging modality, the authors compared 18F-FDG hrPET and MRI detection of pituitary adenomas in Cushing disease (CD). METHODS Consecutive patients with CD who underwent preoperative 18F-FDG hrPET and MRI (spin echo [SE] and spoiled gradient recalled [SPGR] sequences) were prospectively analyzed. Standardized uptake values (SUVs) were calculated from hrPET and were compared with MRI findings. Imaging findings were correlated to operative and histological findings. RESULTS Ten patients (7 females and 3 males) were included (mean age 30.8 ± 19.3 years; range 11–59 years). MRI revealed a pituitary adenoma in 4 patients (40% of patients) on SE and 7 patients (70%) on SPGR sequences. 18F-FDG hrPET demonstrated increased 18F-FDG uptake consistent with an adenoma in 4 patients (40%; adenoma size range 3–14 mm). Maximum SUV was significantly higher for 18F-FDG hrPET–positive tumors (difference = 5.1, 95% CI 2.1–8.1; p = 0.004) than for 18F-FDG hrPET–negative tumors. 18F-FDG hrPET positivity was not associated with tumor volume (p = 0.2) or dural invasion (p = 0.5). Midnight and morning ACTH levels were associated with 18F-FDG hrPET positivity (p = 0.01 and 0.04, respectively) and correlated with the maximum SUV (R = 0.9; p = 0.001) and average SUV (R = 0.8; p = 0.01). All 18F-FDG hrPET–positive adenomas had a less than a 180% ACTH increase and 18F-FDG hrPET–negative adenomas had a greater than 180% ACTH increase after CRH stimulation (p = 0.03). Three adenomas were detected on SPGR MRI sequences that were not detected by 18F-FDG hrPET imaging. Two adenomas not detected on SE (but no adenomas not detected on SPGR) were detected on 18F-FDG hrPET. CONCLUSIONS While 18F-FDG hrPET imaging can detect

  9. Genetics Home Reference: Cushing disease

    Science.gov (United States)

    ... and in the face. Affected individuals may get stretch marks (striae) on their thighs and abdomen and bruise ... to weakness and reduced stamina, thinning skin causing stretch marks and easy bruising, thinning of the bones resulting ...

  10. Approach to the patient with persistent acromegaly after pituitary surgery.

    Science.gov (United States)

    Katznelson, Laurence

    2010-09-01

    The approach to a patient with acromegaly and persistent disease after surgery requires a complex diagnostic assessment. Acromegaly is a chronic and insidious disease that is associated with multisystem comorbidities, including cardiovascular disease, hypertension, sleep apnea syndrome, colon polyposis, arthropathy, and metabolic complications including glucose intolerance and type 2 diabetes mellitus. Patients also have a variety of signs and symptoms, including headache, arthralgias, carpal tunnel syndrome, sweating, fatigue, and psychological issues that impact significantly on quality of life. The recommended approach to the evaluation of the postoperative patient includes a biochemical assessment, with measurement of serum IGF-I along with a glucose-suppressed GH value, radiological assessment to determine location of residual tumor and presence of mass effects, a physical examination for evidence of skeletal and soft tissue overgrowth and related signs of acromegaly, and a thorough clinical assessment for the presence of comorbidities. Repeat surgery is indicated if there is residual tumor that is surgically accessible and there may be a chance for surgical cure, or if there are persistent mass effects upon the optic chiasm. Otherwise, medical therapy is indicated, utilizing somatostatin analogs, dopamine agonists, and pegvisomant, a GH receptor antagonist. Radiation therapy is usually relegated to situations where medical therapy is ineffective or poorly tolerated or where patients would prefer not to sustain the cost of long-term medical therapy. The choice of therapy requires close dialog among endocrinologists, neurosurgeons, radiation therapists, and neuroophthalmologists for optimal care of patients.

  11. Morphologic study of microcirculation in acromegaly by capillaroscopy.

    Science.gov (United States)

    Schiavon, F; Maffei, P; Martini, C; De Carlo, E; Fais, C; Todesco, S; Sicolo, N

    1999-09-01

    Although wide range investigations on the heart and great vessels have been reported in acromegaly, the field of microcirculation is still largely vacant. The nailfold is a window through which we can observe in vivo the vascular bed. This study investigates through nailfold capillaroscopy the morphology of cutaneous microcirculation in acromegaly in relationship with the usual hormonal parameters of disease activity. Twenty-five acromegalic patients and 26 normal subjects, age and sex matched, were studied. A subgroup of acromegalics (8 patients) was considered in stable remission, and the remaining 17 had active disease. Capillaroscopy was performed in each subject by in vivo computer aided stereomicroscopy (magnification, x400). The following morphological parameters were calculated: the number of tortuous loops, meandering capillaries, and capillaries per millimeter; avascular areas; visibility of subpapillary plexus; the capillary length; and intercapillary distance. We were unable to perform the exam in 4 of 25 patients because visibility was poor. The capillary number and length were significantly reduced in acromegalics compared to controls [8.9 +/- 1.5 vs. 10.3 +/- 1.2 no./mm (P = 0.0010) and 174 +/- 49 vs. 255 +/- 24 microm (P acromegaly, morphological alterations also affect the peripheral microcirculation, which seems to be influenced by the activity of the disease. We believe that nailfold capillaroscopy may represent an additional useful tool in the follow-up of acromegalic patients.

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

    Science.gov (United States)

    2016-10-10

    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

  13. Pasireotide in Acromegaly: A Review.

    Science.gov (United States)

    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.

  14. The surgical treatment of acromegaly.

    Science.gov (United States)

    Buchfelder, Michael; Schlaffer, Sven-Martin

    2017-02-01

    Surgical extraction of as much tumour mass as possible is considered the first step of treatment in acromegaly in many centers. In this article the potential benefits, disadvantages and limitations of operative acromegaly treatment are reviewed. Pertinent literature was selected to provide a review covering current indications, techniques and results of operations for acromegaly. The rapid reduction of tumour volume is an asset of surgery. To date, in almost all patients, minimally invasive, transsphenoidal microscopic or endoscopic approaches are employed. Whether a curative approach is feasible or a debulking procedure is planned, can be anticipated on the basis of preoperative magnetic resonance imaging. The radicality of adenoma resection essentially depends on localization, size and invasive character of the tumour. The normalization rates of growth hormone and IGF-1 secretion, respectively, depend on tumour-related factors such as size, extension, the presence or absence of invasion and the magnitude of IGF-1 and growth hormone oversecretion. However, also surgeon-related factors such as experience and patient load of the centers have been shown to strongly affect surgical results and the rate of complications. As compared to most medical treatments, surgery is relatively cheap since the costs occur only once and not repeatedly. There are several new technical gadgets which aid in the surgical procedure: navigation and variants of intraoperative imaging. For the mentioned reasons, current algorithms of acromegaly management suggest an initial operation, unless the patients are unfit for surgery, refuse an operation or only an unsatisfactory resection is anticipated. A few suggestions are made when a re-operation could be considered.

  15. ASSESSMENT OF DIASTOLIC DYSFUNCTION, ARTERIAL STIFFNESS, AND CAROTID INTIMA-MEDIA THICKNESS IN PATIENTS WITH ACROMEGALY.

    Science.gov (United States)

    Cansu, Güven Barış; Yılmaz, Nusret; Yanıkoğlu, Atakan; Özdem, Sebahat; Yıldırım, Aytül Belgi; Süleymanlar, Gültekin; Altunbaş, Hasan Ali

    2017-05-01

    Early diagnosis and treatment of cardiovascular diseases, the most frequent cause of morbidity and mortality in acromegaly, may be an efficient approach to extending the lifespan of affected patients. Therefore, it is crucial to determine any cardiovascular diseases in the subclinical period. The study objectives were to determine markers of subclinical atherosclerosis and asses heart structure and function. This was a cross-sectional, single-center study of 53 patients with acromegaly and 22 age- and sex-matched healthy individuals. Carotid intima-media thickness (CIMT), pulse-wave velocity (PWV), and echocardiographic data were compared between these groups. CIMT and PWV were higher in the acromegaly group than in the healthy group (P = .008 and P = .002, respectively). Echocardiography showed that left ventricular diastolic dysfunction was present in 11.3% of patients. Left ventricular mass index and left atrial volume index were higher in the patients (P = .016 and Pacromegaly and the control group. Our results showed that subclinical atherosclerosis (i.e., CIMT and PWV markers) and heart structure and function were worse in patients with acromegaly than in healthy individuals. Because there were no differences in these parameters between patients with controlled and uncontrolled acromegaly, our results suggest that the structural and functional changes do not reverse with biochemical control. AA = active acromegaly BSA = body surface area CA = biochemically controlled acromegaly CH = concentric hypertrophy CIMT = carotid intima-media thickness DBP = diastolic blood pressure DM = diabetes mellitus ECHO = echocardiography EDV = enddiastolic volume EF = ejection fraction ESV = endsystolic volume GH = growth hormone HC = healthy control HL = hyperlipidemia HT = hypertension IGF-1 = insulin-like growth factor 1 LA = left atrial LAV = left atrial volume LAVI = left atrial volume index LV = left ventricular LVDD = left ventricular diastolic dysfunction LVEF = left

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

    Directory of Open Access Journals (Sweden)

    Christofides EA

    2016-07-01

    Full Text Available Elena A Christofides Endocrinology Associates, Inc., Columbus, OH, USA Abstract: In acromegaly, achieving biochemical control (growth hormone [GH] level <1.0 ng/mL and age- and sex-normalized levels of insulin-like growth factor 1 [IGF-1] through timely diagnosis and appropriate treatment provides an opportunity to improve patient outcomes. Diagnosis of acromegaly is challenging because it is rooted in observing subtle clinical manifestations, and it is typical for acromegaly to evolve for up to 10 years before it is recognized. This results in chronic exposure to elevated levels of 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. Keywords: disease burden, growth hormone, insulin-like growth factor 1, medical therapy, pituitary

  17. Acromegaly and associated tumours: what should gastroenterologists know?

    Science.gov (United States)

    Calderón, María Del Rosario; Delgado, Elvira; García Campos, Francisco

    2017-01-01

    Acromegaly is a clinical syndrome caused by the excessive production of growth hormone. It is associated with high morbidity and significantly increased mortality, mainly due to cardiovascular and respiratory complications, and cancer. Mortality is reduced to that of the general population following successful treatment, in other words, when insulin-like growth factor (IGF-I) and growth hormone values return to normal levels. Not all tumours associated with this syndrome benefit from cost-effective early diagnosis programmes. An in-depth knowledge on the part of clinicians of the morbidity and mortality associated with acromegaly, allowing them in many cases to anticipate the expected clinical course of the disease, is the best therapeutic and follow-up strategy in these patients. Copyright © 2016 Elsevier España, S.L.U., AEEH y AEG. All rights reserved.

  18. Growth hormone deficiency in treated acromegaly.

    Science.gov (United States)

    Mazziotti, Gherardo; Marzullo, Paolo; Doga, Mauro; Aimaretti, Gianluca; Giustina, Andrea

    2015-01-01

    Growth hormone deficiency (GHD) of the adult is characterized by reduced quality of life (QoL) and physical fitness, skeletal fragility, and increased weight and cardiovascular risk. Hypopituitarism may develop in patients after definitive treatment of acromegaly, but an exact prevalence of GHD in this population is still uncertain owing to limited awareness and the scarce and conflicting data available on this topic. Because acromegaly and GHD may yield adverse consequences on similar target systems, the final outcomes of some complications of acromegaly may be further affected by the occurrence of GHD. However, it is still largely unknown whether patients with post-acromegaly GHD may benefit from GH replacement. We review the diagnostic, clinical, and therapeutic aspects of GHD in adult patients treated for acromegaly. Copyright © 2014 Elsevier Ltd. All rights reserved.

  19. Development of acromegaly in patients with prolactinomas

    DEFF Research Database (Denmark)

    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 hyperprolactina......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...

  20. Development of acromegaly in patients with prolactinomas

    DEFF Research Database (Denmark)

    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 hyperprolactina......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...

  1. A case of juvenile acromegaly that was initially diagnosed as severe congestive heart failure from acromegaly-induced dilated cardiomyopathy.

    Science.gov (United States)

    Sue, Mariko; Yoshihara, Aya; Okubo, Yoichiro; Ishikawa, Mayumi; Ando, Yasuyo; Hiroi, Naoki; Shibuya, Kazutoshi; Yoshino, Gen

    2010-01-01

    Acromegaly is characterized by chronic hypersecretion of growth hormone (GH) and is associated with increased mortality rate because of the potential complications such as cardiovascular disease, respiratory disease, or malignancy, which are probably caused by the long-term exposure of tissues to excess GH, for at least 10 years, before diagnosis and treatment. A 22-year-old man with a 2-month history of fatigue was admitted to our hospital because of chest discomfort, dyspnea, and pitting edema of the lower limbs experienced over a 1-month period. On admission, his height and body weight were 186 cm and 138.5 kg, respectively, with a BMI of 39.8 kg/m(2). He showed acromegalic features and elevated serum GH and IGF-1 levels, which were 11.5 ng/mL and 960 ng/mL, respectively. There was no GH suppression in the 75-g oral glucose tolerance test. Pituitary magnetic resonance imaging (MRI) revealed microadenoma. Chest X-ray revealed cardiomegaly, and echocardiogram showed dilated left ventricular (LV) cavity and diffuse hypokinesis with extremely decreased ejection fraction (EF). He was diagnosed as having acromegaly with congestive heart failure from diastolic cardiomyopathy. After the successful transsphenoidal resection of the pituitary adenoma, the level of GH was normalized. However, the cardiac dysfunction did not show any improvement even after the administration of β-blockers, angiotensin-converting enzyme inhibitor (ACE-I), or diuretics. The patient was re-hospitalized, and he died of cardiac failure at the age of 25 years. Patients with acromegaly have been reported to have about 30% higher mortality rate, and cardiovascular disease accounts for 60% of the deaths. We report a case of a patient with juvenile acromegaly who was diagnosed with severe cardiac failure at the time of diagnosis and failed to recover cardiac function even after the successful resection of the pituitary adenoma. Immediate diagnosis and treatment are required for better control of

  2. Geographical information system (GIS) as a new tool to evaluate epidemiology based on spatial analysis and clinical outcomes in acromegaly

    OpenAIRE

    Naves, Luciana Ansaneli; Porto, Lara Benigno; Rosa, João Willy Corrêa; Casulari, Luiz Augusto; Rosa, José Wilson Corrêa

    2013-01-01

    Geographical information systems (GIS) have emerged as a group of innovative software components useful for projects in epidemiology and planning in Health Care System. This is an original study to investigate environmental and geographical influences on epidemiology of acromegaly in Brazil. We aimed to validate a method to link an acromegaly registry with a GIS mapping program, to describe the spatial distribution of patients, to identify disease clusters and to evaluate if the access to Hea...

  3. Cushing's ulcer: the eponym and his own.

    Science.gov (United States)

    Wijdicks, Eelco F M

    2011-06-01

    One of the least remembered eponyms associated with Harvey Cushing is "Cushing's ulcer." The basis of this credit is a paper published in 1932 in which Cushing describes patients who postoperatively and unexpectedly died of perforated peptic ulcers. It is one of the first descriptions of a stress ulcer and a treatise on the brain-stomach connection. Harvey Cushing was puzzled by the pathogenesis of these peptic ulcerations and perforations and advanced several theories. The least plausible included the bile-vomiting theory suggesting that hemorrhagic ulceration could be produced by a combination of bile and acid in a patient recovering from the anesthetic. Other theories were stimulation of a parasympathetic center in the diencephalon or a disturbance of vagal centers in the brainstem. Quite surprisingly to Cushing, the Boston Herald implicitly insinuated that Cushing found the cause of ulcers and this claim upset him greatly. It is ironic that Harvey Cushing, in his later years with failing health, developed an ulcer himself. Cushing noted in his correspondence that he felt the agitation over this newspaper clipping caused his later ulcer. The first description of a neurogenic ulcer remains an important medical observation and is a testament to Cushing's broad accomplishments.

  4. Gammaknife radiosurgery in patients with acromegaly.

    Science.gov (United States)

    Erdur, Fatih M; Kilic, Türker; Peker, Selcuk; Celik, Ozlem; Kadioglu, Pinar

    2011-12-01

    We aimed to evaluate the efficacy and reliability of gamma-knife radiosurgery (GKR) in 22 patients with acromegaly at the Endocrinology-Metabolism Clinic of Cerrahpasa Medical School. We collected data retrospectively from hospital records on disease activity and other pituitary functions, pituitary MRI and visual fields, before GKR and 6, 12, 24, 36, 48 and 60 months after GKR. The median follow-up duration after GKR was 60 months (interquartile range [IQR]: 24-60 months). The remission rate was 54.5% after the 60 months of follow-up. The median growth hormone (GH) level at 60 months after GKR (0.99 ng/mL [IQR: 0.36-2.2]) was significantly lower than the median GH level before GKR (5.65 ng/mL [IQR: 3.85-7.2] (p=0.002). The median insulin-like growth factor-1 (IGF-1) level 60 months after GKR (221.5 ng/mL [IQR: 149-535]) was significantly lower than the median IGF-1 level before GKR (582.5 ng/mL [IQR: 515-655]) (p=0.008). Tumour growth was well controlled in 20 patients (95.2%). Six patients (28.6%) developed new-onset hypopituitarism. We concluded that GKR is an effective adjuvant treatment to control tumour growth, lower GH and IGF-1 levels, and to increase remission rates in patients with acromegaly who were refractory to surgical and medical treatment. Copyright © 2011 Elsevier Ltd. All rights reserved.

  5. Acromegaly in a patient with a pulmonary neuroendocrine tumor: case report and review of current literature.

    Science.gov (United States)

    Krug, Sebastian; Boch, Michael; Rexin, Peter; Pfestroff, Andreas; Gress, Thomas; Michl, Patrick; Rinke, Anja

    2016-06-27

    Pulmonary neuroendocrine tumors (NET) form a heterogeneous group of rare diseases. In these tumors, paraneoplastic syndromes have been described to drive the course of the disease, among them acromegaly induced by paraneoplastic secretion of growth hormone-releasing hormone (GHRH). We report the case of a 43 years old patient initially diagnosed with acromegaly accompanied by weight gain and acral enlargement. Subsequently, further diagnostic work-up identified a solitary pulmonary neuroendocrine tumor (NET). Laboratory tests revealed markedly increased growth hormone (GH) and insulin-like growth factor 1 (IGF-1) without GHRH elevation in the absence of pituitary pathologies confirming the paraneoplastic origin of clinical presentation with acromegaly. Curative surgery was performed leading to normalization of the elevated hormone levels and improvement of the clinical symptoms. Immunohistochemically, a typical carcinoid (TC) was seen with low proliferation index and abundant IGF-1 expression. The association of acromegaly and pulmonary NET has only rarely been reported. We present an individual case of paraneoplastic GH- and IGF-1 secretion in a patient with pulmonary NET. Based on their rarity, the knowledge of paraneoplastic syndromes occurring in patients with pulmonary NET such as acromegaly due to paraneoplastic GH- and IGF-1 secretion is mandatory to adequately diagnose and treat these patients.

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

    Energy Technology Data Exchange (ETDEWEB)

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

    2007-11-15

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

  7. Carotid artery protrusion and dehiscence in patients with acromegaly.

    Science.gov (United States)

    Sasagawa, Yasuo; Tachibana, Osamu; Doai, Mariko; Hayashi, Yasuhiko; Tonami, Hisao; Iizuka, Hideaki; Nakada, Mitsutoshi

    2016-10-01

    Acromegaly is a systemic disease which causes multiple bony alterations. Some authors reported that acromegalic patients have risk factors for an intraoperative vascular injury due to the specific anatomical features of their sphenoid sinus. The objective of our study was to analyze the anatomic characteristics of sphenoid sinus in acromegalic patients compared with controls, by evaluation of computed tomography (CT) findings. We examined 45 acromegalic (acromegaly group) and 45 non-acromegalic patients (control group) with pituitary adenomas who were matched for sex, age, height, tumor size, and cavernous sinus invasion (Knosp grade). Preoperative CT of the pituitary region including the sphenoid sinus was used to evaluate the following anatomic characteristics: type of sphenoid sinus (sellar or pre-sellar/conchal); intrasphenoid septa (non/single or multiple); carotid artery protrusion; carotid artery dehiscence; intercarotid distance. Sixteen acromegalic patients (35.5 %) and 6 controls (13.3 %) had carotid artery protrusion. Additionally, 10 acromegalic patients (22.2 %) and 3 controls (6.6 %) had carotid artery dehiscence. Carotid artery protrusion and dehiscence were more frequent in the acromegaly group than in control group (p = 0.013 and 0.035, respectively). Other anatomic characteristics (type of sphenoid sinus, intrasphenoid septa, and intracarotid distance) showed no significant differences between acromegaly and control groups. Our study suggests that carotid artery protrusion and dehiscence occur more frequently among acromegalic patients, compared with non-acromegalic patients. It is important for surgeons to be aware of these anatomic variations to avoid vital complications, such as carotid injuries, during surgery.

  8. Management of acromegaly in Latin America: expert panel recommendations.

    Science.gov (United States)

    Barkan, Ariel; Bronstein, Marcello D; Bruno, Oscar D; Cob, Alejandro; Espinosa-de-los-Monteros, Ana Laura; Gadelha, Monica R; Garavito, Gloria; Guitelman, Mirtha; Mangupli, Ruth; Mercado, Moisés; Portocarrero, Lesly; Sheppard, Michael

    2010-06-01

    Although there are international guidelines orienting physicians on how to manage patients with acromegaly, such guidelines should be adapted for use in distinct regions of the world. A panel of neuroendocrinologists convened in Mexico City in August of 2007 to discuss specific considerations in Latin America. Of major discussion was the laboratory evaluation of acromegaly, which requires the use of appropriate tests and the adoption of local institutional standards. As a general rule to ensure diagnosis, the patient's GH level during an oral glucose tolerance test and IGF-1 level should be evaluated. Furthermore, to guide treatment decisions, both GH and IGF-1 assessments are required. The treatment of patients with acromegaly in Latin America is influenced by local issues of cost, availability and expertise of pituitary neurosurgeons, which should dictate therapeutic choices. Such treatment has undergone profound changes because of the introduction of effective medical interventions that may be used after surgical debulking or as first-line medical therapy in selected cases. Surgical resection remains the mainstay of therapy for small pituitary adenomas (microadenomas), potentially resectable macroadenomas and invasive adenomas causing visual defects. Radiotherapy may be indicated in selected cases when no disease control is achieved despite optimal surgical debulking and medical therapy, when there is no access to somatostatin analogues, or when local issues of cost preclude other therapies. Since not all the diagnostic tools and treatment options are available in all Latin American countries, physicians need to adapt their clinical management decisions to the available local resources and therapeutic options.

  9. The cardiovascular phenotype of a mouse model of acromegaly.

    Science.gov (United States)

    Izzard, Ashley S; Emerson, Michael; Prehar, Sukhpal; Neyses, Ludwig; Trainer, Peter; List, Edward O; Kopchick, John J; Heagerty, Anthony M

    2009-10-01

    Although, it is accepted that there is an excess of cardiovascular mortality in acromegaly, it is uncertain whether this is due to the direct effects of growth hormone-induced-cardiomyopathy or is a consequence of atherosclerosis secondary to the metabolic syndrome often observed in this condition. Direct comparison of a mouse model of acromegaly to a mouse model of Laron's syndrome allowed us to carry out detailed phenotyping and better understand the role GH plays in the circulatory system. Transgenic mice that overexpress the growth hormone gene (GH) developed gigantism, including insulin resistance and higher blood pressures commensurate with increased body mass. In these giant mice, the hearts were hypertrophied but haemodynamic studies suggested contractile function was normal. Segments of small arteries mounted in a pressure myograph showed vascular wall hypertrophy but a preserved lumen diameter. Vascular contractile function was normal. Mice in which the GH receptor gene was disrupted or 'knocked out' were dwarf and had low blood pressure, small hearts and blood vessels but a normally functioning circulation. Correlations of body mass with cardiovascular parameters suggested that blood pressure and structural characteristics develop in line with body size. In this transgenic mouse model of acromegaly, there is cardiac and vascular hypertrophy commensurate with GH excess but normal function. Our findings support the contention that the excess mortality in this condition may be due to the development of hypertrophic cardiomyopathy rather than increased rates of atherosclerotic coronary artery disease.

  10. Pathologies of the skin and its appendages in endocrine diseases

    Directory of Open Access Journals (Sweden)

    Hubert Arasiewicz

    2016-05-01

    Full Text Available Patients suffering from endocrine disorders often present a wide profile of skin lesions. In hyperthyroidism we observe hair loss, lower leg myxedema and onycholysis or, in the case of hormone deficiency, generalized swelling of the skin, which becomes cold and pale. Primary hyperparathyroidism is revealed by pruritus, presence of chronic urticaria or deposition of amorphous calcium salts. In hypoparathyroidism, the skin is dry while the nails become very brittle. Skin lesions in diabetes include necrobiosis lipoidica, granuloma annulare, scleroderma-like diabetic edema and acanthosis nigricans. Overactive pituitary gland is often manifested as acromegaly with hypertrophy of soft tissue thickening and hypertrichosis. The skin in the early stages of hypopituitarism feels swollen, is pale yellow and oily, and finally becomes alabaster and dry. The characteristic features of Cushing syndrome are central obesity, lunar face, buffalo hump, and striae. In Addison’s disease we observe hyperpigmentation. Hyperandrogenism in women leads to acne, hirsutism and virilization.

  11. Accelerated Telomere Shortening in Acromegaly; IGF-I Induces Telomere Shortening and Cellular Senescence

    Science.gov (United States)

    Matsumoto, Ryusaku; Fukuoka, Hidenori; Iguchi, Genzo; Odake, Yukiko; Yoshida, Kenichi; Bando, Hironori; Suda, Kentaro; Nishizawa, Hitoshi; Takahashi, Michiko; Yamada, Shozo; Ogawa, Wataru; Takahashi, Yutaka

    2015-01-01

    Objective Patients with acromegaly exhibit reduced life expectancy and increased prevalence of age-related diseases, such as diabetes, hypertension, and cardiovascular disease. However, the underlying mechanism has not been fully elucidated. Telomere shortening is reportedly associated with reduced life expectancy and increased prevalence of these age-related diseases. Methods We measured telomere length in patients with acromegaly using quantitative PCR method. The effect of GH and IGF-I on telomere length and cellular senescence was examined in human skin fibroblasts. Results Patients with acromegaly exhibited shorter telomere length than age-, sex-, smoking-, and diabetes-matched control patients with non-functioning pituitary adenoma (0.62 ± 0.23 vs. 0.75 ± 0.35, respectively, P = 0.047). In addition, telomere length in acromegaly was negatively correlated with the disease duration (R2 = 0.210, P = 0.003). In vitro analysis revealed that not GH but IGF-I induced telomere shortening in human skin fibroblasts. Furthermore, IGF-I-treated cells showed increased senescence-associated β-galactosidase activity and expression of p53 and p21 protein. IGF-I-treated cells reached the Hayflick limit earlier than GH- or vehicle-treated cells, indicating that IGF-I induces cellular senescence. Conclusion Shortened telomeres in acromegaly and cellular senescence induced by IGF-I can explain, in part, the underlying mechanisms by which acromegaly exhibits an increased morbidity and mortality in association with the excess secretion of IGF-I. PMID:26448623

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

    Science.gov (United States)

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

    2016-01-01

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

  13. Does Apolipoprotein E genotype affect cardiovascular risk in subjects with acromegaly?

    Science.gov (United States)

    Bozok Cetintas, Vildan; Zengi, Ayhan; Tetik, Asli; Karadeniz, Muammer; Ergonen, Faruk; Kucukaslan, Ali Sahin; Tamsel, Sadik; Kosova, Buket; Sahin, Serap Baydur; Saygılı, Fusun; Eroglu, Zuhal

    2012-06-01

    Acromegaly is a syndrome that results when the pituitary gland produces excess growth hormone after epiphyseal closure at puberty. Usually, subjects with acromegaly exhibit a 2- to 3-fold higher mortality rate from diseases that are associated with cardiovascular complications when compared to the normal population. In this study, we therefore aimed to evaluate whether a well-established cardiovascular risk factor, the Apolipoprotein E (Apo E) genotype, contributes to increased risk of cardiovascular complications in subjects with acromegaly. A total of 102 unrelated acromegaly subjects were prospectively included into this case-control association study and constituted our study group. The study group was comparable by age and gender with 200 unrelated healthy subjects constituting our control group. Genomic DNA was isolated from the peripheral blood leukocytes of all subjects and Apo E genotype (codon 112/158) was assessed by melting temperature analyses after using a real-time PCR protocol. The Apolipoprotein E4 allele was found at a significantly higher frequency in the study group when compared with the control group (P = 0.032). Subjects with the E2 allele, on the other hand, had significantly increased values in body mass index (P = 0.004), waist circumference (P = 0.001), C-reactive protein (CRP) (P acromegaly since it is concurrently present with other cardiovascular risk factors such as the left-side carotid intima media thickness and CRP.

  14. Acromegaly presenting as hirsuitism: Uncommon sinister aetiology of a common clinical sign

    Directory of Open Access Journals (Sweden)

    Rajesh Jain

    2012-01-01

    Full Text Available Hirsuitism though not uncommon (24%, is not considered to be a prominent feature of acromegaly because of its lack of specificity and occurrence. Hirsuitism is very common in women of reproductive age (5-7% and has been classically associated with polycystic ovarian syndrome (PCOS. Twenty-eight year lady with 3 year duration of hirsuitism (Modified Ferriman Gallwey score-24/36 , features of insulin resistance (acanthosis, subtle features of acromegaloidism (woody nose and bulbous lips was diagnosed to have acromegaly in view of elevated IGF-1 (1344 ng/ml; normal: 116-358 ng/ml, basal (45.1 ng/ml and post glucose growth hormone (39.94 ng/ml and MRI brain showing pituitary macroadenoma. Very high serum androstenedione (>10 ng/ml; normal 0.5-3.5 ng/ml, elevated testosterone (0.91 ng/ml, normal <0.8 and normal dehydroepiandrosterone sulphate (DHEAS (284 mcg/dl, normal 35-430 mcg/dl along with polycystic ovaries on ultrasonography lead to diagnosis of associated PCOS. She was also diagnosed to have diabetes. This case presentation intends to highlight that hirsuitism may rarely be the only prominent feature of acromegaly. A lookout for subtle features of acromegaly in all patients with hirsuitism and going for biochemical evaluation (even at the risk of investigating many patients of insulin resistance and acromegloidism may help us pick up more patients of acromegaly at an earlier stage thus help in reducing disease morbidity.

  15. Rab18 is reduced in pituitary tumors causing acromegaly and its overexpression reverts growth hormone hypersecretion.

    Science.gov (United States)

    Vazquez-Martinez, Rafael; Martinez-Fuentes, Antonio J; Pulido, Marina R; Jimenez-Reina, Luis; Quintero, Ana; Leal-Cerro, Alfonso; Soto, Alfonso; Webb, Susan M; Sucunza, Nuria; Bartumeus, Frederic; Benito-Lopez, Pedro; Galvez-Moreno, Maria A; Castaño, Justo P; Malagon, Maria M

    2008-06-01

    Rab proteins regulate the sequential steps of intracellular membrane transport. Alterations of these GTPases and their associated proteins are emerging as the underlying cause for several human diseases involving dysregulated secretory activities. Herein we investigated the role of Rab18, which negatively regulates hormone secretion by interacting with secretory granules, in relation to the altered functioning of tumoral pituitary somatotropes causing acromegaly. A total of 18 patients diagnosed with pituitary tumors causing acromegaly (nine patients) or nonfunctioning adenomas (nine patients) underwent endoscopic transsphenoidal surgery. Adenomas were subsequently processed to evaluate Rab18 production in relation to GH secretion. We found that somatotropinoma cells are characterized by a high secretory activity concomitantly with a remarkably reduced Rab18 expression (15%) and protein content levels (30%), as compared with cells from nonfunctioning pituitary adenomas derived from patients with normal or reduced GH plasma levels (100%). Furthermore, immunoelectron microscopy revealed that Rab18 association with the surface of GH-containing secretory granules was significantly lower in somatotropes from acromegalies than nonfunctioning pituitary adenomas. Finally, we provide evidence that modulation of Rab18 gene expression can revert substantially the hypersecretory activity of cells because Rab18 overexpression reduced by 40% the capacity of cells from acromegalies to respond to GHRH stimulation. These results suggest that molecular alterations affecting individual components of the secretory granule traffic machinery can contribute to maintain a high level of GH in plasma. Accordingly, Rab18 constitutes a valuable target as a diagnostic, prognostic, and/or therapeutic tool for human acromegaly.

  16. MJA Practice Essentials--Endocrinology. 9: Pituitary disease in adults.

    Science.gov (United States)

    Hurley, David M; Ho, Ken K Y

    2004-04-19

    Pituitary adenomas are found in 10%-25% of unselected autopsy series and are evident in about 10% of asymptomatic individuals by magnetic resonance imaging. Diagnosis of pituitary disorders is often delayed by lack of awareness and the subtlety of symptoms and signs. Hypopituitarism is suspected when peripheral hormone concentrations are low without an elevation in the corresponding pituitary tropic hormone(s). Severe adult-onset growth-hormone deficiency results in reduced muscle mass, increased fat mass and diminished quality of life, which are reversed by growth hormone replacement therapy. While trans-sphenoidal surgery remains first-line treatment for acromegaly, drug treatment has an important role in controlling residual growth-hormone excess and, in some circumstances, as first-line treatment. Dopamine-agonist therapy (cabergoline or bromocriptine) is the treatment of choice for micro- and macroprolactinomas. In patients with suggestive clinical features, elevated 24-hour urine free cortisol level is usually sufficient to diagnose endogenous Cushing's syndrome; careful additional investigation is needed to determine whether the cause is Cushing's disease (pituitary adenoma secreting adrenocorticotropic hormone [ACTH]), ectopic ACTH secretion or adrenal disease. Heightened awareness is needed to detect the sometimes subtle symptoms and signs of pituitary disease

  17. 临床多学科综合治疗团队诊治库欣病%Efficacy analysis of diagnosis and treatment of Cushing's disease with a multidisciplinary team

    Institute of Scientific and Technical Information of China (English)

    张绿; 王镛斐; 叶红英; 张朝云; 赵曜; 葛亮; 姚振威; 汪寅; 潘力

    2015-01-01

    目的 分析临床多学科综合治疗团队(MDT)诊治库欣病的疗效及经验.方法 回顾性纳入2013年1月至12月复旦大学附属华山医院在MDT诊治流程模式下诊治的45例库欣综合征患者.其中22例行双侧岩下窦采血,确诊库欣病40例(初诊断32例,首次治疗后复发或未缓解8例).大腺瘤、微腺瘤和MRI阴性分别为8、26、6例,3例为侵袭性;33例接受神经外科手术(经鼻手术32例,开颅手术1例).总结其诊断、治疗情况.结果 33例接受手术的患者中,术后1周缓解者22例(66.7%),其术前、术后1周内血皮质醇最低值差异有统计学意义[分别为(869±361)、(60±41)nmol/L,P<0.01].术后3个月总体缓解25例(76%);大腺瘤、微腺瘤和MRI阴性及侵袭性垂体瘤缓解的比例分别为6/6、14/18、3/6、2/3.病理学显示,27例促肾上腺皮质激素瘤、6例增生或腺组织者分别有25例(92.6%)和0例缓解.手术后未缓解的8例中,2例纳入帕瑞肽新药研究、3例行伽玛刀治疗、1例开始甲吡酮治疗、1例待再次手术治疗、1例失访.7例患者出现手术并发症.结论 MDT模式下多学科参与的病例诊治有助于患者及时选择最佳的治疗方案,提高治疗效果.%Objective To analyze the efficacy and experiences of the diagnosis and treatment of Cushing' s disease with a multidisciplinary team.Methods Forty-five patients with Cushing' s syndrome treated under the MDT diagnosis and treatment process mode at Shanghai Huashan Hospital,Fudan University from January to December 2013 were analyzed retrospectively.Among the 45 patients,22 underwent bilateral inferior petrosal sinus blood sampling,and 40 were diagnosed as Cushing' s syndrome.Macroadenomas,microadenomas,and MRI negative were 8,26,and 6,respectively,3 of them were invasive.Thirty-two patients were newly diagnosed,8 relapsed or did not relieve after the first treatment.Their diagnosis,management,efficacy,and room for improvement were summarized

  18. Comparison between total and subtotal adrenalectomy procedures for recurrent Cushing' s disease%不同肾上腺切除方式治疗复发性库欣病疗效分析

    Institute of Scientific and Technical Information of China (English)

    丁雪飞; 李汉忠; 周广臣

    2011-01-01

    目的 探讨不同肾上腺切除方法治疗复发性库欣病临床效果.方法 经垂体治疗后库欣病复发患者32例,男7例,女25例,平均年龄(31.9±10.4)岁.随机分为两组:18例患者右侧肾上腺全切+左侧次全切除(次全切组);14例双侧肾上腺切除(全切组).结果 术后随访18~60个月,32例患者术后24 h游离皮质醇降至正常范围,81.5%(22/27)高血压、65.4%(17/26)糖尿病或糖耐量异常患者术后血压、血糖降至正常范围,66.7%(12/18)女性闭经患者月经恢复正常,两组间血压、血糖及月经恢复情况无统计学差异.次全切除组1例术后复发,5例患者无需补充糖皮质激素;全切组14.3%(2/14)出现Nelson综合征.结论 肾上腺全切和肾上腺次全切除是治疗库欣病复发的两种有效方法.%Objective To discuss clinical efficacy of different techniques to resect adrenal gland for recurrent Cushing's disease. Methods We reviewed the cases (32 patients, 7 were men and 25 were women) of recurrent Cushing's disease postoperatively with a mean age of (31. 9 ± 10. 4 ) years. It included 18 cases of total right adrenalectomy and left subtotal adrenalectomy ( subtotal resection group ) and 14 cases bilateral total adrenalectomy (total resection group ) . Results Hypercortisolism disappeared after surgery and the 24 hours UFC decreased within the normal range. 81.5% of those with hypertension and 65. 4% of those with diabetes mellitus or impaired glucose tolerance showed normal blood pressure and blood glucose respectively and 66. 7% female returned to normal menstrual cycle after operation. There was no significant difference of normalization of blood pressure , blood glucose and menstrual cycle between two groups . One case with hypercortisolism recurrence of the subtotal resection group was cured by the resection of the residual gland. All patients of the total resection group required glucocorticoid replacement therapy , while 5 cases of subtotal resection

  19. The quality of life and psychological, social and cognitive functioning of patients with acromegaly.

    Science.gov (United States)

    Szcześniak, Dorota; Jawiarczyk-Przybyłowska, Aleksandra; Rymaszewska, Joanna

    2015-01-01

    Acromegaly is a rare and chronic disease, most often caused by a pituitary adenoma. Excessive secretion of the growth hormone (GH) leads to hepatic secretion of insulin-like growth factor-1 (IGF-1), which in turn causes characteristic changes in the patient's appearance, many skeletal deformities and metabolic disorders. In addition to somatic symptoms, acromegalic patients demonstrate psychosocial and personality deficits, as well as common co-occurrence of mental disorders. There are few studies investigating acromegaly in Poland. In recent years, the concept of quality of life has become fundamental to understanding health problems. Studies dealing with acromegaly likewise tend to include assessments of quality of life of patients suffering from this endocrinopathy.

  20. Complications of acromegaly: thyroid and colon.

    Science.gov (United States)

    Tirosh, Amit; Shimon, Ilan

    2017-02-01

    In acromegaly the long-term exposure to high growth hormone (GH) and insulin-like growth factor-1 (IGF-1) levels may result in specific complications in different human organs, including the thyroid gland and the colon. We will review here the evidence available regarding the characteristic thyroid and colon complications in acromegaly. This review summarizes the published data observing noncancerous structural abnormalities (thyroid nodules, colonic polyps) and thyroid and colon cancer in patients diagnosed with acromegaly. Thyroid micro-carcinomas are probably over-diagnosed among acromegalic patients. In regard to colon cancer, there is no sufficient data to suggest that colon cancer risk is higher in acromegaly compared to the general population.

  1. Megaduodenum in a patient with acromegaly

    Directory of Open Access Journals (Sweden)

    Belinda George

    2012-01-01

    Full Text Available Introduction: Acromegaly is associated with enlargement of all organs including the gastro intestinal system. However, there are no previous reports of occurrence of megaduodenum in patients with acromegaly. Discussion: We present the case of a 47 year old male who was diagnosed to have acromegaly 13 years ago and had undergone transsphenoidal surgery twice with incomplete removal of the pituitary macro-adenoma and received radiotherapy following the second surgery. Patient has been on replacement therapy for hypocortisolism, hypothyroidism and hypogonadism since 10 years. Post glucose growth hormone level continued to remain unsuppressed; however, patient never received any medical therapy for acromegaly. He was evaluated with esophago-gastro-duodenoscopy for recurrent abdominal pain and distension, which showed an elongated and tortuous megaduodenum. These findings were verified with a barium study which revealed dilated stomach, first and second part of duodenum with no evidence of a distal obstruction. Conclusions: We report this finding in view of the rare association.

  2. Diagnosis and clinical genetics of Cushing syndrome in pediatrics

    Science.gov (United States)

    Stratakis, Constantine A.

    2016-01-01

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

  3. Kleine-Levin and Munchausen syndromes in a patient with recurrent acromegaly.

    Science.gov (United States)

    Jungheim, K; Badenhoop, K; Ottmann, O G; Usadel, K H

    1999-02-01

    Hypothalamic disease often affects the patients' personality and this also applies to pituitary tumors with suprasellar extension. We report on a patient with a 12-year history of recurrent acromegaly, treated with three transphenoidal operations, single field radiation therapy and bromocriptine/octreotide administration. During the course of follow-up she presented with self-inflicted anemia and Kleine-Levin syndrome (hypersomnia, hyperphagia and hypersexuality). Furthermore, she developed post-radiation necrosis within the right temporal lobe. Whether her neurological and personality disorders result - at least partially - from the acromegaly or the temporal lobe necrosis remains unclear.

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

    Science.gov (United States)

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

    2015-06-01

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

  5. Comorbidities, treatment patterns and cost-of-illness of acromegaly in Sweden: a register-linkage population-based study.

    Science.gov (United States)

    Lesén, Eva; Granfeldt, Daniel; Houchard, Aude; Dinet, Jérôme; Berthon, Anthony; Olsson, Daniel S; Björholt, Ingela; Johannsson, Gudmundur

    2017-02-01

    Acromegaly is a complex endocrine disease with multiple comorbidities. Treatment to obtain biochemical remission includes surgery, medical therapy and radiation. We aimed to describe comorbidities, treatment patterns and cost-of-illness in patients with acromegaly in Sweden. A nationwide population-based study. Patients with acromegaly were identified and followed in national registers in Sweden. Longitudinal treatment patterns were assessed in patients diagnosed between July 2005 and December 2013. The cost-of-illness during 2013 was estimated from a societal perspective among patients diagnosed between 1987 and 2013. Among 358 patients with acromegaly (48% men, mean age at diagnosis 50.0 (s.d. 15.3) years) at least one comorbidity was reported in 81% (n = 290). The most common comorbidities were hypertension (40%, n = 142), neoplasms outside the pituitary (30%, n = 109), hypopituitarism (22%, n = 80) and diabetes mellitus (17%, n = 61). Acromegaly treatment was initiated on average 3.7 (s.d. 6.9) months after diagnosis. Among the 301 treated patients, the most common first-line treatments were surgery (60%, n = 180), somatostatin analogues (21%, n = 64) and dopamine agonists (14%, n = 41). After primary surgery, 24% (n = 44) received somatostatin analogues. The annual per-patient cost was €12 000; this was €8700 and €16 000 if diagnosed before or after July 2005, respectively. The cost-of-illness for acromegaly and its comorbidities was 77% from direct costs and 23% from production loss. The prevalence of comorbidity is high in patients with acromegaly. The most common first-line treatment in acromegalic patients was surgery followed by somatostatin analogues. The annual per-patient cost of acromegaly and its comorbidities was €12 000. © 2017 European Society of Endocrinology.

  6. Pachydermoperiostosis: a rare mimicker of acromegaly

    Directory of Open Access Journals (Sweden)

    Noor Rafhati Adyani Abdullah

    2017-05-01

    Full Text Available Pachydermoperiostosis is a very rare osteoarthrodermopathic disorder whose clinical and radiographic presentations may mimic those of acromegaly. In the evaluation of patients with acromegaloid appearances, pachydermoperiostosis should be considered as a differential diagnosis. In this article, we report a 17-year-old boy who presented with 2-year history of acral enlargement and facial appearance changes associated with joint pain and excessive sweating. He had been investigated extensively for acromegaly, and the final diagnosis was pachydermoperiostosis.

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

    Science.gov (United States)

    Sharma, Susmeeta T

    2017-06-01

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

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

    Science.gov (United States)

    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 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.

  9. Congestive heart failure in acromegaly: A review of 6 cases

    OpenAIRE

    Dutta, P.; Das, S; Bhansali, A; S K Bhadada; B V Rajesh; Reddy, K. S.; Vaiphei, K; K. K. Mukherjee; Pathak, A.; Shah, V N

    2012-01-01

    Background: Though cardiac involvement is common in acromegaly, overt congestive heart failure is uncommon. Materials and Methods: This is retrospective analysis of hospital record between 1996 and 2007. We analyzed records of 150 consecutive patients with acromegaly. We included the patients with acromegaly those who had overt congestive heart failure either at presentation or during the course of illness for the present analysis. The diagnosis of acromegaly and congestive cardiac failure we...

  10. Cushing Syndrome: Other FAQs

    Science.gov (United States)

    ... Kronenberg, H. M., Shlomo, M., Polonsky, K. S., Larsen P. R. (Eds.). Williams textbook of endocrinology (12th ed.). (chap. 15). Philadelphia, PA: Saunders Elsevier. [top] Abraham, M. R., & Smith, C. V. Adrenal disease and pregnancy. Retrieved April ...

  11. Subclinical Cushing syndrome: a review.

    Science.gov (United States)

    Starker, Lee F; Kunstman, John W; Carling, Tobias

    2014-06-01

    Owing to its diagnostic challenges, subclinical Cushing syndrome (SCS) is likely to be highly underdiagnosed and undertreated, and the overall incidence may be as high as 5% to 20% in patients with adrenal incidentalomas. The diagnosis can be established by a systematic and thorough biochemical evaluation. SCS has been associated with significant morbidity, which at least partly may be reversed by surgery. Given the low rates of complications and the possibility to reverse the detrimental effects of elevated cortisol secretion, minimally invasive adrenalectomy is recommended for patients with biochemically proven or suspected SCS who are appropriate surgical candidates. Copyright © 2014 Elsevier Inc. All rights reserved.

  12. A rare cause of acromegaly: McCune-Albright syndrome

    Directory of Open Access Journals (Sweden)

    Erdal Bodakçi

    2015-06-01

    Full Text Available McCune-Albright syndrome is characterized by polyostatic fibrous dysplasia, brown spots on the skin (café au lait pigmentation and autonomous endocrine hyperfunction. Early puberty and other endocrinological manifestations, such as acromegaly, gigantism and hypercortisolism are widely observed in the syndrome. Acromegaly is seen in 20% of patients. We report a case of acromegaly accompanied with this syndrome.

  13. Prevalence of diabetes mellitus in patients with acromegaly

    NARCIS (Netherlands)

    Dreval, A V; Trigolosova, I V; Misnikova, I V; Kovalyova, Y A; Tishenina, R S; Barsukov, I A; Vinogradova, A V; Wolffenbuttel, B H R

    2014-01-01

    Early carbohydrate metabolism disorders (ECMDs) and diabetes mellitus (DM) are frequently associated with acromegaly. We aimed to assess the prevalence of ECMDs in patients with acromegaly and to compare the results with those in adults without acromegaly using two population-based epidemiologic

  14. Prevalence of diabetes mellitus in patients with acromegaly

    NARCIS (Netherlands)

    Dreval, A V; Trigolosova, I V; Misnikova, I V; Kovalyova, Y A; Tishenina, R S; Barsukov, I A; Vinogradova, A V; Wolffenbuttel, B H R

    2014-01-01

    Early carbohydrate metabolism disorders (ECMDs) and diabetes mellitus (DM) are frequently associated with acromegaly. We aimed to assess the prevalence of ECMDs in patients with acromegaly and to compare the results with those in adults without acromegaly using two population-based epidemiologic sur

  15. Self-perception of cognitive function among patients with active acromegaly, controlled acromegaly, and non-functional pituitary adenoma: a pilot study.

    Science.gov (United States)

    Yedinak, Chris G; Fleseriu, Maria

    2014-08-01

    Pituitary adenomas (PAs) represent 15 % of all brain tumors. One-sixth of these are reported to cause acromegaly via excess growth hormone secretion. These tumors have been associated with multiple comorbidities, including neuropsychiatric and cognitive dysfunction. We aimed to assess patient perception of cognitive deficits and the relationship of cognitive changes to active acromegaly (AA) versus controlled acromegaly (CA) versus non-functional PAs (NFPA). A modified FACT-Cog survey was used, which focused on the prevalence and severity of perceived dysfunction in five areas of cognitive function: ability to learn, concentration/distractibility, mental agility, memory and recall, and verbal recall. Patient perception of current health and health change over the previous 12 months was also assessed. The overall perceived prevalence and severity of cognitive dysfunction were the highest among NFPA groups, particularly in the areas of mental agility, verbal recall, and memory/recall. Patients with AA reported greater prevalence and severity of dysfunction with respect to concentration/distractibility and ability to learn. Patients with AA reported the best overall current health, though patients with CA reported the greatest improvement in health over the previous year. These findings may indicate that PAs can affect cognitive function regardless of whether excess growth hormone is present. Acromegaly and NFPA patients perceive specific areas of cognitive dysfunction that may require further evaluation and treatment. Further research may be useful regarding patient quality of life, patient functionality during normal daily activities, and perceived dysfunction despite biological disease control.

  16. Increased prevalence of gallbladder polyps in acromegaly.

    Science.gov (United States)

    Annamalai, Anand K; Gayton, Emma L; Webb, Alison; Halsall, David J; Rice, Caiomhe; Ibram, Ferda; Chaudhry, Afzal N; Simpson, Helen L; Berman, Laurence; Gurnell, Mark

    2011-07-01

    Several studies have suggested an increased prevalence of benign and malignant tumors in acromegaly, particularly colonic neoplasms. The gallbladder's epithelial similarity to the colon raises the possibility that gallbladder polyps (GBP) may occur more frequently in acromegaly. Thirty-one patients with newly diagnosed acromegaly (14 females, 17 males; mean age 54.7 yr, range 27-76 yr) were referred to our center between 2004 and 2008. All had pituitary adenomas and were treated with somatostatin analogs prior to transsphenoidal surgery. Biliary ultrasonography was performed at the time of referral. In a retrospective case-cohort study, we compared the prevalence of GBP in these scans with those of 13,234 consecutive patients (age range 20-80 yr) presenting at the hospital for abdominal/biliary ultrasound during the same time interval. Associations between GH and IGF-I levels and GBP in acromegaly were also examined. There was a higher prevalence of GBP in patients with acromegaly compared with controls (29.03 vs 4.62%, P = 0.000008); relative risk was 6.29 (95% confidence interval 3.61-10.96). Eight of nine patients with acromegaly and GBP were older than 50 yr of age. GH levels were higher in those with GBP (median 30.8 μg/liter, interquartile range 10.9-39.1) than those without (8.2 μg/liter, interquartile range 6.0-16.0), but IGF-I levels were comparable. This is the first study to demonstrate an increased prevalence of GBP in patients with newly diagnosed acromegaly. Further studies are required to determine whether these patients are at increased risk of developing gallbladder carcinoma and to define the role, if any, of biliary ultrasound surveillance.

  17. Acromegaly and McCune-Albright Syndrome

    Science.gov (United States)

    Salenave, Sylvie; Boyce, Alison M.; Collins, Michael T.

    2014-01-01

    Background: McCune-Albright syndrome (MAS) includes the triad of poly/monostotic fibrous dysplasia, café-au-lait spots, and hyperfunctioning endocrinopathies. Acromegaly affects around 20% of MAS patients. Aims: The objective was to review all reported cases of acromegaly associated with MAS. Methods: All studies and case reports of acromegaly in patients with MAS were systematically sought in the world literature up to January 2013. We also included new data (from three unreported cases) and updated data on 23 previously reported patients from our two centers. Results: We reviewed the cases of 112 patients (65 males). Mean age at diagnosis of acromegaly was 24.4 years (range, 3–64). Among the 40 pediatric patients, 23 (57%) had precocious puberty. GH/IGF-1 excess was suggested by accelerated growth in 85% of pediatric cases. Acromegaly was almost always associated with skull base fibrous dysplasia. Modern imaging techniques (computed tomography or magnetic resonance imaging) revealed an adenoma in 54% of the patients (macroadenoma in more than two-thirds). Median GH levels and mean IGF-1 SD score at diagnosis were 57 μg/L (2.8 to 291 μg/L) and 8 (2.3 to 24), respectively. Hyperprolactinemia was present in 81% (mean, 149 μg/L; range, 21–600). Pituitary surgery, performed in 25 cases, very rarely cured the GH/IGF-1 excess. Somatostatin analogs improved GH/IGF-1 levels in most patients but achieved control of acromegaly in only 17 (30%) of 56 patients. Pegvisomant achieved normal IGF-1 levels in 10 of 13 cases. Conclusion: Acromegaly, which is present in 20–30% of patients with MAS, raises particular diagnostic and therapeutic issues. PMID:24517150

  18. A hyperkinetic heart in uncomplicated active acromegaly. Explanation of hypertension in acromegalic patients?

    Science.gov (United States)

    Thuesen, L; Christensen, S E; Weeke, J; Orskov, H; Henningsen, P

    1988-01-01

    Cardiac function was studied by echocardiography in 12 patients with active acromegaly and in 12 age- and sex-matched healthy control subjects. None of the patients had cardiovascular diseases or other endocrine diseases than acromegaly. The patients had a mean age of 39 +/- 5 years and were short-term acromegalic with a mean duration of disease of 6 +/- 3 years. Mean left ventricular mass was 163 +/- 43 g/m2 in the acromegalic group versus 120 +/- 24 g/m2 in the control group. Preload (the diastolic diameter of the left ventricle) was within normal limits, while afterload (end-systolic meridional wall stress) was significantly decreased in the acromegalic group. Myocardial contractility assessed as fractional shortening of the left ventricle was 39.9 +/- 3.6% in the acromegalic group versus 32.9 +/- 5.1% in the control group, and cardiac output was increased by 52% in the acromegalic group because of increased heart rate and stroke volume. We suggest that augmented peripheral blood flow is responsible for the condition of cardiac hyperkinesia in short-term acromegaly and involved in the development of hypertension, which is a frequent complication of long-term acromegaly.

  19. The changing face of acromegaly--advances in diagnosis and treatment.

    Science.gov (United States)

    Ribeiro-Oliveira, Antônio; Barkan, Ariel

    2012-10-01

    Acromegaly is a chronic disease characterized by the presence of a pituitary growth hormone (GH)-producing tumour, excessive secretion of growth hormone, raised levels of insulin-like growth factor I (IGF-I) and characteristic clinical presentation of acral enlargement. Over the past two decades, major advances have occurred in the understanding of some aspects of acromegaly--such as the biology of pituitary tumours, the physiology, molecular mechanisms of GH secretion and IGF-I generation, and the pathogenesis of comorbidities. Moreover, new approaches to diagnosis and surveillance (both in terms of screening and follow-up) of acromegaly have led to increases in the number of patients diagnosed with active disease, many of whom would previously have been missed. The development of sensitive assays for detecting plasma GH and IGF-I levels, as well as the widespread use of MRI for visualization of small tumours, have been major contributing factors to these improvements. Treatment advances have resulted in improved cure rates and disease control through novel neurosurgical techniques and pharmacological approaches. This Review summarizes and discusses the changes in our understanding of the epidemiology, diagnosis, treatment, and follow-up of acromegaly and its comorbidities.

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

    DEFF Research Database (Denmark)

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

    2001-01-01

    , 5.06; CI, 1.86–11.0). In patients with adrenal adenoma, SMR was 3.95 (CI, 0.81–11.5). The perceived quality of health was significantly impaired only in patients with Cushing’s disease and appeared independent of disease control or presence of hypopituitarism. It is concluded that 1) Cushing...

  1. The Modern Criteria for Medical Management of Acromegaly.

    Science.gov (United States)

    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.

  2. Bone material strength index as measured by impact microindentation is altered in patients with acromegaly.

    Science.gov (United States)

    Malgo, F; Hamdy, N A T; Rabelink, T J; Kroon, H M; Claessen, K M J A; Pereira, A M; Biermasz, N R; Appelman-Dijkstra, N M

    2017-03-01

    Acromegaly is a rare disease caused by excess growth hormone (GH) production by the pituitary adenoma. The skeletal complications of GH and IGF-1 excess include increased bone turnover, increased cortical bone mass and deteriorated microarchitecture of trabecular bone, associated with a high risk of vertebral fractures in the presence of relatively normal bone mineral density (BMD). We aimed to evaluate tissue-level properties of bone using impact microindentation (IMI) in well-controlled patients with acromegaly aged ≥18 years compared to 44 controls from the outpatient clinic of the Centre for Bone Quality. In this cross-sectional study, bone material strength index (BMSi) was measured in 48 acromegaly patients and 44 controls with impact microindentation using the osteoprobe. Mean age of acromegaly patients (54% male) was 60.2 years (range 37.9-76.5), and 60.5 years (range 39.8-78.6) in controls (50% male). Patients with acromegaly and control patients had comparable BMI (28.2 kg/m(2) ± 4.7 vs 26.6 kg/m(2) ± 4.3, P = 0.087) and comparable BMD at the lumbar spine (1.04 g/cm(2) ± 0.21 vs 1.03 g/cm(2) ± 0.13, P = 0.850) and at the femoral neck (0.84 g/cm(2) ± 0.16 vs 0.80 g/cm(2) ± 0.09, P = 0.246). BMSi was significantly lower in acromegaly patients than that in controls (79.4 ± 0.7 vs 83.2 ± 0.7; P acromegaly after reversal of long-term exposure to pathologically high GH and IGF-1 levels. Our findings also suggest that methods other than DXA should be considered to evaluate bone fragility in patients with acromegaly. © 2017 European Society of Endocrinology.

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

    Directory of Open Access Journals (Sweden)

    Marelys Yanes Quesada

    2011-12-01

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

  4. Multiple Myeloma in a Patient with Acromegaly

    Directory of Open Access Journals (Sweden)

    Yu Mi Kang

    2015-03-01

    Full Text Available Acromegaly is a slowly progressing condition resulting from excess growth hormone (GH, generally caused by a GH-secreting pituitary adenoma. Cancer is the third most common cause of mortality in patients with acromegaly, and insulin-like growth factor 1 (IGF-1 is known to influence tumor formation by increasing cell proliferation and inhibiting apoptosis. Multiple myeloma (MM is a plasma cell neoplasm, and previous studies have suggested the possible role of IGF-1 in its development of MM. However, no cases of acromegaly accompanied with MM have been reported in Asia to date. We here report the case of a 58-year-old woman with acromegaly accompanied with MM who presented with longstanding acromegalic manifestations resulting from a GH-secreting pituitary adenoma and also exhibited anemia, a reversed albumin/globulin ratio, and plasmacytosis on bone marrow examination. Because IGF-1 has been suggested to play an important role in the development and progression of MM, the patient promptly underwent surgical removal of the pituitary adenoma via a transsphenoidal approach. Since there is currently no consensus on therapeutic guidelines and suggested prognosis for MM with acromegaly, long-term follow-up of such cases is needed.

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

    Science.gov (United States)

    Starkman, Monica N

    2013-09-01

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

  6. Glucocorticoid- and androgen-secreting black adrenocortical adenomas: unique cause of corticotropin-independent Cushing syndrome.

    Science.gov (United States)

    Tanaka, Satoshi; Tanabe, Akiyo; Aiba, Motohiko; Hizuka, Naomi; Takano, Kazue; Zhang, Jun; Young, William F

    2011-01-01

    To describe the unique association of corticotropin-independent Cushing syndrome caused by cortisol- and androgen-secreting black adrenal cortical adenomas with myelolipomatous change. We report the clinical, laboratory, radiologic, and pathologic findings from 2 patients who presented with androgen excess and typical signs and symptoms of Cushing syndrome. Endocrine investigations showed high serum cortisol concentrations that lacked diurnal rhythm, undetectable plasma corticotropin concentrations, and absence of serum cortisol suppression after overnight dexamethasone suppression tests. Serum levels of adrenal androgens were elevated. Computed tomography of the abdomen revealed unilateral adrenal masses (largest lesional diameters 4.0 and 3.1 cm). On the basis of the plurihormonal hypersecretion and the imaging characteristics, adrenocortical carcinoma was considered as a possible diagnosis. However, histopathologic analysis in both patients revealed black adrenal cortical adenomas with myelolipomatous change. After surgery, adrenal androgens normalized, and the signs and symptoms of Cushing syndrome and androgen excess resolved. There was no evidence of recurrent disease at last follow-up. A unique form of corticotropin-independent Cushing syndrome is described: cortisol- and androgen-secreting black adrenal cortical adenomas with myelolipomatous change. Although most patients with corticotropin-independent Cushing syndrome associated with androgen excess prove to have adrenocortical carcinoma, the clinician should be aware of the possibility of benign, black adrenal adenomas in this clinical setting.

  7. Increased Population Risk of AIP-Related Acromegaly and Gigantism in Ireland.

    Science.gov (United States)

    Radian, Serban; Diekmann, Yoan; Gabrovska, Plamena; Holland, Brendan; Bradley, Lisa; Wallace, Helen; Stals, Karen; Bussell, Anna-Marie; McGurren, Karen; Cuesta, Martin; Ryan, Anthony W; Herincs, Maria; Hernández-Ramírez, Laura C; Holland, Aidan; Samuels, Jade; Aflorei, Elena Daniela; Barry, Sayka; Dénes, Judit; Pernicova, Ida; Stiles, Craig E; Trivellin, Giampaolo; McCloskey, Ronan; Ajzensztejn, Michal; Abid, Noina; Akker, Scott A; Mercado, Moises; Cohen, Mark; Thakker, Rajesh V; Baldeweg, Stephanie; Barkan, Ariel; Musat, Madalina; Levy, Miles; Orme, Stephen M; Unterländer, Martina; Burger, Joachim; Kumar, Ajith V; Ellard, Sian; McPartlin, Joseph; McManus, Ross; Linden, Gerard J; Atkinson, Brew; Balding, David J; Agha, Amar; Thompson, Chris J; Hunter, Steven J; Thomas, Mark G; Morrison, Patrick J; Korbonits, Márta

    2017-01-01

    The aryl hydrocarbon receptor interacting protein (AIP) founder mutation R304(*) (or p.R304(*) ; NM_003977.3:c.910C>T, p.Arg304Ter) identified in Northern Ireland (NI) predisposes to acromegaly/gigantism; its population health impact remains unexplored. We measured R304(*) carrier frequency in 936 Mid Ulster, 1,000 Greater Belfast (both in NI) and 2,094 Republic of Ireland (ROI) volunteers and in 116 NI or ROI acromegaly/gigantism patients. Carrier frequencies were 0.0064 in Mid Ulster (95%CI = 0.0027-0.013; P = 0.0005 vs. ROI), 0.001 in Greater Belfast (0.00011-0.0047) and zero in ROI (0-0.0014). R304(*) prevalence was elevated in acromegaly/gigantism patients in NI (11/87, 12.6%, P acromegaly/gigantism cases. tMRCA is consistent with historical/folklore accounts of Irish giants. Forward simulations predict many undetected carriers; geographically targeted population screening improves asymptomatic carrier identification, complementing clinical testing of patients/relatives. We generated disease awareness locally, necessary for early diagnosis and improved outcomes of AIP-related disease. © 2016 The Authors. **Human Mutation published by Wiley Periodicals, Inc.

  8. Screening for Cushing's syndrome in obese patients

    Directory of Open Access Journals (Sweden)

    Ozay Tiryakioglu

    2010-01-01

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

  9. Cushing's ulcer in traumatic brain injury

    Institute of Scientific and Technical Information of China (English)

    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.

  10. Acne and systemic disease.

    Science.gov (United States)

    Lolis, Margarita S; Bowe, Whitney P; Shalita, Alan R

    2009-11-01

    Acne is the most common disease of the skin. It affects 85% of teenagers, 42.5% of men, and 50.9% of women between the ages of 20 and 30 years.96,97 The role of hormones, particularly as a trigger of sebum production and sebaceous growth and differentiation, is well known. Excess production of hormones, specifically androgens, GH, IGF-1, insulin, CRH, and glucocorticoids, is associated with increased rates of acne development. Acne may be a feature in many endocrine disorders, including polycystic ovary disease, Cushing syndrome, CAH, androgen-secreting tumors, and acromegaly. Other nonendocrine diseases associated with acne include Apert syndrome, SAPHO syndrome, Behçet syndrome and PAPA syndrome. Acne medicamentosa is the development of acne vulgaris or an acneiform eruption with the use of certain medications. These medications include testosterone, progesterone,steroids, lithium, phenytoin, isoniazid, vitamins B2, B6, and B12, halogens, and epidermal growth factor inhibitors. Management of acne medicamentosa includes standard acne therapy. Discontinuation of the offending drug may be necessary in recalcitrant cases. Basic therapeutic interventions for acne include topical therapy, systemic antibiotics,hormonal agents, isotretinoin, and physical treatments. Generally, the severity of acne lesions determines the type of acne regimen necessary. The emergence of drug-resistant P acnes and adverse side effects are current limitations to effective acne management.

  11. Disorders of carbohydrate metabolism and their relationship with the prevalence of cancer in patients with acromegaly – authors’ own observations

    Directory of Open Access Journals (Sweden)

    Joanna Elżbieta Malicka

    2016-09-01

    Full Text Available Background . Acromegaly is a rare chronic disease with an excessive secretion of growth hormone, which leads to characteristic changes in appearance, organ and metabolic complications and neoplasms, which are a significant cause of shortened survival time. One of the most common consequences of acromegaly is disturbed carbohydrate metabolism. Objectives . The aim of this study was to assess the prevalence of disorders of carbohydrate tolerance and their relationship with the prevalence of malignant tumours in subjects with acromegaly. Material and methods. The study group comprised 69 patients with confirmed acromegaly (26 M, 43 F, aged 26–83 (mean 58.9 ± 11.0. Medical histories and the results of laboratory tests – plasma fasting glucose and oral glucose tolerance test, as well as the results of imaging and histopathological examinations performed in cases of suspected cancer lesions were analyzed. Results Disorders of carbohydrate tolerance were confirmed in a total of 46 patients (66.7%. 23 patients (33.3% had normal glucose tolerance. Diabetes was diagnosed in 22 patients (31.9%, while prediabetes was diagnosed in 24 (34.8% patients. In the analyzed group there were 6 cases of cancers, including 5 patients with concomitant diabetes, which represented a value significantly higher (p = 0.01 compared to patients without diabetes. The sixth case of cancer was detected in a woman with IGT . In patients with normal glucose tolerance there was no case of cancer. Conclusions . Among patients with acromegaly, disorders of carbohydrate tolerance occur in more than half of the cases, which confirms the need for active screening in this group of patients, in which a significant role is played by General Practitioners. General Practitioners should also consider the possibility of acromegaly in the differential diagnosis of glucose tolerance disorders detected in their patients. All patients with acromegaly, especially with concomitant diabetes or

  12. Unsuccessful treatment of acromegaly with medroxyprogesterone acetate.

    Science.gov (United States)

    Atkinson, R L; Dimond, R C; Howard, W J; Earll, J M

    1974-09-01

    6 patients with active acromegaly were treated with 10 mg of medroxyprogesterone acetate (MPA) every 6 hours daily for 2 weeks to 6 months. Oral glucose tolerance tests, growth hormone (GH) levels, and insulin tolerance tests (ITT) were done before and during MPA treatment. Basal GH levels varied widely during control and therapy periods; no significant lowering of GH levels occurred during treatment. Carbohydrate tolerance was not significantly affected by MPA therapy, although 5 out of 6 patients had deterioration at least once during treatment. Blood glucose response to ITT was unchanged by MPA. MPA did not affect the clinical features of acromegaly. There was no consistent effect of MPA on insulin-induced or arginine-induced GH secretion. It is concluded that MPA is not an effective agent for treating acromegaly.

  13. Acromegaly

    Science.gov (United States)

    ... leaks and, rarely, meningitis. Cerebrospinal fluid bathes the brain and can leak from the nose if the incision area doesn’t heal well. Meningitis is a bacterial or viral infection of the meninges, the outer covering of the brain. Even when surgery is successful and hormone levels ...

  14. A Case of Primary Hyperparathyroidism Combined with Cushing Syndrome due to Ectopic ACTH Secretion

    Directory of Open Access Journals (Sweden)

    N.T. Rikhsiieva

    2015-03-01

    Full Text Available A rare case of primary hyperparathyroidism (PHPT combined with Cushing syndrome due to ectopic ACTH secretion in 37-year-old women is described. The patient gradually underwent surgeries after compensation of general condition: bilateral parathyroidectomy, in 4 months — removal of carcinoid tumor of the lung. In case of Cushing syndrome or PHPT, the authors recommended to carry out careful examination of patients to exclude MEN-1 and MEN-2 syndromes. In addition, it is necessary to exclude the presence of familial forms of the disease, i.e., it is necessary to carry out a survey of first-degree relatives.

  15. Pachydermoperiostosis: a rare mimicker of acromegaly.

    Science.gov (United States)

    Abdullah, Noor Rafhati Adyani; Jason, Wong Lok Chin; Nasruddin, Azraai Bahari

    2017-01-01

    Pachydermoperiostosis is a very rare osteoarthrodermopathic disorder whose clinical and radiographic presentations may mimic those of acromegaly. In the evaluation of patients with acromegaloid appearances, pachydermoperiostosis should be considered as a differential diagnosis. In this article, we report a 17-year-old boy who presented with 2-year history of acral enlargement and facial appearance changes associated with joint pain and excessive sweating. He had been investigated extensively for acromegaly, and the final diagnosis was pachydermoperiostosis. There is a broad range of differential diagnosis for acromegaloid features such as acromegaly, pseudoacromegaly with severe insulin resistance, Marfan's syndrome, McCune-Albright and a rare condition called pachydermoperiostosis.Once a patient is suspected to have acromegaly, the first step is biochemical testing to confirm the clinical diagnosis, followed by radiologic testing to determine the cause of the excess growth hormone (GH) secretion. The cause is a somatotroph adenoma of the pituitary in over 95 percent of cases.The first step is measurement of a serum insulin-like growth factor 1 (IGF1). A normal serum IGF1 concentration is strong evidence that the patient does not have acromegaly.If the serum IGF1 concentration is high (or equivocal), serum GH should be measured after oral glucose administration. Inadequate suppression of GH after a glucose load confirms the diagnosis of acromegaly.Once the presence of excess GH secretion is confirmed, the next step is pituitary magnetic resonance imaging (MRI).Atypical presentation warrants revision of the diagnosis. This patient presented with clubbing with no gigantism, which is expected in adolescent acromegalics as the growth spurt and epiphyseal plate closure have not taken place yet.

  16. Updates in outcomes of stereotactic radiation therapy in acromegaly.

    Science.gov (United States)

    Gheorghiu, Monica Livia

    2017-02-01

    Purpose Treatment of acromegaly has undergone important progress in the last 20 years mainly due to the development of new medical options and advances in surgical techniques. Pituitary surgery is usually first-line therapy, and medical treatment is indicated for persistent disease, while radiation (RT) is often used as third-line therapy. The benefits of RT (tumor volume control and decreased hormonal secretion) are hampered by the long latency of the effect and the high risk of adverse effects. Stereotactic RT methods have been developed with the aim to provide more precise targeting of the tumor with better control of the radiation dose received by the adjacent brain structures. The purpose of this review is to present the updates in the efficacy and safety of pituitary RT in acromegalic patients, with an emphasis on the new stereotactic radiation techniques. Methods A systematic review was performed using PubMed and articles/abstracts and reviews detailing RT in acromegaly from 2000 to 2016 were included. Results Stereotactic radiosurgery and fractionated stereotactic RT (FSRT) for patients with persistent active acromegaly after surgery and/or during medical therapy provide comparable high rates of tumor control, i.e. stable or decrease in size of the tumor in 93-100% of patients at 5-10 years and endocrinological remission in 40-60% of patients at 5 years. Hypofractionated RT is an optimal option for tumors located near the optic structures, due to its lower toxicity for the optic nerves compared to single-dose radiosurgery. The rate of new hypopituitarism varies from 10 to 50% at 5 years and increases with the duration of follow-up. The risk for other radiation-induced complications is usually low (0-5% for new visual deficits, cranial nerves damage or brain radionecrosis and 0-1% for secondary brain tumors) and risk of stroke may be higher in FSRT. Conclusion Although the use of radiotherapy in patients with acromegaly has decreased with advances in

  17. Cost-effectiveness of lanreotide Autogel in treatment algorithms of acromegaly.

    Science.gov (United States)

    Biermasz, Nienke R; Roelfsema, Ferdinand; Pereira, Alberto M; Romijn, Johannes A

    2009-06-01

    The introduction of effective pharmacological treatments has changed the management of acromegaly. However, chronic, life-long treatment with somatostatin analogues and/or growth hormone receptor antagonists is very expensive. We estimated the costs of treatment algorithms to control acromegaly from a Dutch perspective. We used the following assumptions: after the diagnosis of acromegaly there is a mean remaining lifespan of approximately 33 years; the success rates of surgery and somatostatin analogues in controlling the disease are approximately 60%; and the lifelong costs of different algorithms to control acromegaly in 100 patients ranged from 43 million euros (primary surgery and secondary somatostatin analogues) to 57 million euros (primary somatostatin analogues and secondary surgery) and even reached 95 million euros (medical treatment only). In algorithms that include trans-sphenoidal surgery, the lifetime treatment costs are almost 46-59% cheaper per 100 patients than in algorithms with medical treatment but without trans-sphenoidal surgery. Algorithms with primary surgery and secondary somatostatin analogs are 30% cheaper per 100 patients than algorithms with primary somatostatin analogues and secondary surgery. Per 100 patients, algorithms including lanreotide Autogel are 14-34% more expensive than algorithms including octreotide long-acting release. These life-long costs should be taken into consideration when making choices between treatment algorithms.

  18. Impact of successful treatment of acromegaly on overnight heart rate variability and sleep apnea.

    Science.gov (United States)

    Chemla, Denis; Attal, Pierre; Maione, Luigi; Veyer, Anne-Sophie; Mroue, Ghassan; Baud, Dany; Salenave, Sylvie; Kamenicky, Peter; Bobin, Serge; Chanson, Philippe

    2014-08-01

    Successful treatment of acromegaly improves disease-related cardiovascular mortality and morbidity, but its effects on autonomic modulation of the heart rate are unknown. We documented treatment-induced changes in time-domain heart rate variability, taking into account the confounding effects of obstructive sleep apnea. Sixteen consecutive patients (12 males, aged 43 ± 12 y) with newly diagnosed acromegaly underwent overnight (12:00-7:00 am) cardiac Holter recordings coupled with polysomnography. Data were obtained before and 10 ± 6 months after successful treatment of acromegaly. IGF-1 levels fell from 807 ± 333 to 207 ± 69 μg/L and normalized in all patients. Seven patients (44%) had obstructive sleep apnea (apnea-hypopnea index 33 ± 21/h) at baseline. Treatment had no significant effect on polysomnographic indices. After treatment, increases were noted in the normal-to-normal heart period (NN), SD-NN, the percentage of NN differing from the previous NN by greater than 50 msec, and the root mean square of successive differences in NN (each P rate variability results were not influenced by the type of treatment, and there was no relationship between changes in NN and changes in the apnea-hypopnea index (P = .58). Early after successful treatment of acromegaly, we observed increased parasympathetic modulation/decreased sympathetic modulation of the nighttime heart rate, an effect that seems unrelated to changes in sleep apnea status. Treatments aimed at normalizing IGF-1 may improve cardiovascular homeostasis through improved cardiac autonomic nervous system modulation.

  19. Patients cured of acromegaly do not experience improvement of their skull deformities.

    Science.gov (United States)

    Rick, Jonathan W; Jahangiri, Arman; Flanigan, Patrick M; Aghi, Manish K

    2017-04-01

    Acromegaly is a rare disease that is associated with many co-morbidities. This condition also causes progressive deformity of the skull which includes frontal bossing and cranial thickening. Surgical and/or medical management can cure this condition in many patients, but it is not understood if patients cured of acromegaly experience regression of their skull deformities. We performed a retrospective analysis on patients treated at our dedicated pituitary center from 2009 to 2014. We looked at all MRI images taken during the treatment of these patients and recorded measurements on eight skull dimensions. We then analyzed these measurements for changes over time. 29 patients underwent curative treatment for acromegaly within our timeframe. The mean age for this population was 45.0 years old (range 19-70) and 55.2 % (n = 16) were female. All of these patients were treated with a transsphenoidal resection for a somatotropic pituitary adenoma. 9 (31.1%) of these patients required further medical therapy to be cured. We found statically significant variation in the coronal width of the sella turcica after therapy, which is likely attributable to changes from transsphenoidal surgery. None of the other dimensions had significant variation over time after cure. Patients cured of acromegaly should not expect natural regression of their skull deformities. Our study suggests that both frontal bossing and cranial thickening do not return to normal after cure.

  20. High Prolidase Levels may be a Marker of Irreversible Extracellular Matrix Changes in Controlled Acromegaly Patients?

    Science.gov (United States)

    Tabur, S; Sezen, H; Korkmaz, H; Ozkaya, M; Akarsu, E

    2016-02-01

    The present study aimed to evaluate the activity of prolidase in controlled acromegaly patients and its association with oxidative stress. 25 acromegalic patients in remission who were followed in our outpatient clinic and 31 healthy controls were enrolled in the study. Serum growth hormone (GH), insulin-like growth factor 1 (IGF-1), total antioxidative status (TAS), total oxidative stress (TOS), total free sulfhydryl (-SH), paraoxonase (PON), arylesterase (ARE), lipid hydroperoxide (LOOH) and prolidase activity levels were measured. Percent ratio of TOS to TAS level was accepted as oxidative stress index (OSI). Serum prolidase activity, TOS, OSI, and LOOH levels were significantly higher in acromegaly patients compared to the healthy control group (pacromegaly patients compared to the healthy control group (p=0.002). Prolidase activity were positively correlated with TOS, OSI, LOOH and negatively correlated with -SH in patients with acromegaly (r=0.471, pacromegaly patients. These results suggest that extracellular matrix changes continue eventhough the disease is controlled, and elevated oxidative stress is involved in the increased prolidase activity in acromegaly patients.

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

    Science.gov (United States)

    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 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. PMID:27366708

  2. Analysis of the clinical characteristics of patients with different gender in Cushing's disease:296 cases in a single center%不同性别库欣病患者的临床特点差异分析:单中心296例临床总结

    Institute of Scientific and Technical Information of China (English)

    张毅; 姚勇; 卢琳; 邓侃; 代从新; 冯铭; 包新杰; 幸兵; 连伟; 王任直

    2015-01-01

    目的:总结垂体促肾上腺皮质激素腺瘤在不同性别之间临床特点的差异。方法回顾性总结2008年3月~2013年12月期间在北京协和医院收治的296例库欣病的临床资料。根据性别分组,分析不同性别组的一般状况、临床表现、体征、激素水平、实验室检查及肿瘤大小等方面的差异。结果库欣病男女患者性别比例为1∶4.92。男性发病年龄(28.34±10.87)岁,女性发病年龄(33.78±12.26)岁,差异无统计学意义( P =0.21)。男性患者在高血压、皮肤薄、紫纹、色素沉着、视力下降、视野缺损和骨质疏松及骨折的发生率明显高于女性患者(P <0.05)。女性患者的血促肾上腺皮质激素、清晨血皮质醇水平高于男性患者,男女库欣病患者的丙氨酸氨基转移酶水平均高于正常值1.6~2.0倍。男女患者肿瘤均以微腺瘤为主。结论垂体促肾上腺皮质激素腺瘤男性患者多于女性,部分临床表现与体征、激素水平、存在在不同性别患者之间存在差异。%Objective To explore clinical characteristics of patients with different gender in Cushing's disease .Methods Clinical data of 296 patients with Cushing's disease were collected from March 2008 to December 2013 and divided into male group and female group in Peking Union Hospital . The gender differences of patients with Cushing's disease were statistically analyzed in demography ,clin‐ical manifestation ,endocrine features laboratory tests and tumor size .Results Sex ratio for patients with Cushing's disease for men and women was 1∶4 .92 .There was no statistically significant differences in the age of presentation between genders (the mean age at presentation was 28 .34 ± 10 .87 years old for male and 33 .78 ± 12 .26 years old for female ,P> 0 .05) .The sexual differences of most common clinical features are statistically different , such as hypertension , skin

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

    Directory of Open Access Journals (Sweden)

    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.

  4. INCIDENCE AND PREVALENCE OF ACROMEGALY IN THE UNITED STATES: A CLAIMS-BASED ANALYSIS.

    Science.gov (United States)

    Broder, Michael S; Chang, Eunice; Cherepanov, Dasha; Neary, Maureen P; Ludlam, William H

    2016-11-01

    Acromegaly, a rare endocrine disorder, results from excessive growth hormone secretion, leading to multisystem-associated morbidities. Using 2 large nationwide databases, we estimated the annual incidence and prevalence of acromegaly in the U.S. We used 2008 to 2013 data from the Truven Health MarketScan(®) Commercial Claims and Encounters Database and IMS Health PharMetrics healthcare insurance claims databases, with health plan enrollees acromegaly (International Classification of Diseases, 9th Revision, Clinical Modification Code [ICD-9CM] 253.0), or 1 claim with acromegaly and 1 claim for pituitary tumor, pituitary surgery, or cranial stereotactic radiosurgery. Annual incidence was calculated for each year from 2009 to 2013, and prevalence in 2013. Estimates were stratified by age and sex. Incidence was up to 11.7 cases per million person-years (PMPY) in MarketScan and 9.6 cases PMPY in PharMetrics. Rates were similar by sex but typically lowest in ≤17 year olds and higher in >24 year olds. The prevalence estimates were 87.8 and 71.0 per million per year in MarketScan and PharMetrics, respectively. Prevalence consistently increased with age but was similar by sex in each database. The current U.S. incidence of acromegaly may be up to 4 times higher and prevalence may be up to 50% higher than previously reported in European studies. Our findings correspond with the estimates reported by a recent U.S. study that used a single managed care database, supporting the robustness of these estimates in this population. Our study indicates there are approximately 3,000 new cases of acromegaly per year, with a prevalence of about 25,000 acromegaly patients in the U.S. CT = computed tomography GH = growth hormone IGF-1 = insulin-like growth factor 1 ICD-9-CM Code = International Classification of Diseases, 9th Revision, Clinical Modification Codes MRI = magnetic resonance imaging PMPY = per million person-years.

  5. Activation of the dopamine receptor type-2 (DRD2) promoter by 9-cis retinoic acid in a cellular model of Cushing's disease mediates the inhibition of cell proliferation and ACTH secretion without a complete corticotroph-to-melanotroph transdifferentiation.

    Science.gov (United States)

    Occhi, Gianluca; Regazzo, Daniela; Albiger, Nora Maria; Ceccato, Filippo; Ferasin, Sergio; Scanarini, Massimo; Denaro, Luca; Cosma, Chiara; Plebani, Mario; Cassarino, Maria Francesca; Mantovani, Giovanna; Stalla, Günter K; Pecori Giraldi, Francesca; Paez-Pareda, Marcelo; Scaroni, Carla

    2014-09-01

    Cushing's disease (CD) is a rare condition in which hypercortisolemia is secondary to excessive ACTH release from a pituitary corticotroph adenoma. CD is associated with significant morbidity and mortality, and a safe therapy that effectively targets the pituitary tumor is still lacking. Retinoic acid (RA) and dopamine agonists (DAs) have recently been considered as monotherapy in CD patients, and satisfactory results have been reported, albeit in a limited number of patients. Given the permissive role of RA on the dopamine receptor type-2 (DRD2), the aim of the present study was to see whether a combination of 9-cis RA and the DA bromocriptine (Br) might represent a possible treatment for CD. Here we show that 9-cis RA induces a functional DRD2 in the pituitary corticotroph cell line AtT20, and increases cell sensitivity to Br via a mechanism only partially related to corticotroph-to-melanotroph transdifferentiation. In addition, 9-cis RA and Br act synergistically to modulate cell viability, with favorable implications for clinical use. In nearly 45% of corticotropinoma-derived primary cultures, the combined administration of 9-cis RA and Br lowered the steady-state level of the ACTH precursor proopiomelanocortin (POMC) more efficiently than either of the drugs alone. In conclusion, the effects of a combination of 9-cis RA and Br on ACTH synthesis/secretion and cell viability in AtT20, and on POMC transcriptional activity in human corticotropinomas might represent a suitable starting point for assessing the potential of this treatment regimen for ACTH-secreting pituitary adenomas. This study thus has potentially important implications for novel therapeutic approaches to CD.

  6. Diabetic ketoacidosis in a patient with acromegaly, a case report

    Directory of Open Access Journals (Sweden)

    O. Akha

    2007-01-01

    Full Text Available Abstract Although impaired glucose tolerance affects 36% and overt diabetes mellitus can be seen in 30% of cases with acromegaly, diabetic ketoacidosis is rarely reported in patients with this disease. We present an unusual complication of acromegaly: a 22 year old woman with amenorrhea (6 month ago and blurred vision of left eye (4 month ago referred to Ghaem neurology clinic in Mashhad. With a growth hormone (GH level of =100ng/ml, Insulin like growth factor-1 (IGF-1 =1560 ng/ml and pituitary macro adenoma in magnetic resonance imaging (MRI, she was candidated for surgery. A few hours before surgery she had dyspnea, loss of consciousness, fever and with blood sugar (BS =500 mg/ml, Hco3=2.4, PH=7.06 and keton in urine. Diabetic ketoacidosis was diagnosed. The patient was treated with high dose of insulin (500 IU/day and hydration. After octerotide administration the need for insulin decreased and after pituitary adenectomy, there was no need for insulin therapy and the patient was discharged on metformin (one tablet per day.

  7. The Acylated/Unacylated Ghrelin Ratio Is Similar in Patients With Acromegaly During Different Treatment Regimens.

    Science.gov (United States)

    Muhammad, Ammar; Delhanty, Patric J D; Huisman, Martin; Visser, Jenny A; Jan van der Lelij, Aart; Neggers, Sebastian J C M M

    2017-07-01

    Data on plasma acylated ghrelin (AG) and unacylated ghrelin (UAG) levels in acromegaly are limited. High AG/UAG ratios are linked with type 2 diabetes, obesity, and hyperphagia (e.g., in Prader-Willi syndrome). To assess fasting plasma AG and UAG levels, and the AG/UAG ratio in acromegaly patients receiving combination treatment of long-acting somatostatin analogs (LA-SSAs) and pegvisomant (PEGV; n = 60). We used as controls acromegaly patients whose disease was controlled with PEGV monotherapy and medically naïve patients with active acromegaly. Fasting venous blood samples were collected and directly stabilized to inhibit deacylation of AG. Plasma AG and UAG levels were determined by double-antibody sandwich enzyme immunoassay, and the AG/UAG ratio was calculated. Plasma AG and UAG levels were significantly lower in patients with acromegaly receiving combination treatment [median, interquartile range (IQR): AG: 8.5 pg/mL, 2.9 to 21.1 pg/mL; UAG: 26.9 pg/mL, 11.2 to 42.1 pg/mL] compared with patients using PEGV alone [AG: 60.5 pg/mL (IQR, 58.8 to 77.4 pg/mL); UAG: 153.7 pg/mL (IQR, 127.3 to 196.0 pg/mL)] and medically naïve patients with acromegaly [AG: 24.0 pg/mL (IQR, 12.6 to 49.7 pg/mL); UAG: 56.3 pg/mL (IQR, 43.4 to 61.5 pg/mL)]. However, AG/UAG ratios were similar in all groups. Although plasma AG and UAG are suppressed during combination treatment with LA-SSAs and PEGV, the AG/UAG ratio remained similar. This shows that SSAs decrease both AG and UAG levels, which suggests that they do not alter metabolism significantly in acromegaly patients.

  8. PREDICTORS OF QUALITY OF LIFE IN 165 PATIENTS WITH ACROMEGALY: RESULTS FROM A SINGLE-CENTER STUDY.

    Science.gov (United States)

    Kreitschmann-Andermahr, Ilonka; Buchfelder, Michael; Kleist, Bernadette; Kohlmann, Johannes; Menzel, Christa; Buslei, Rolf; Kołtowska-Häggsträm, Maria; Strasburger, Christian; Siegel, Sonja

    2017-01-01

    Even if treated, acromegaly has a considerable impact on patient quality of life (QoL); despite this, the exact clinical determinants of QoL in acromegaly are unknown. This study retrospectively examines a cohort of treated patients with acromegaly, with the aim of identifying these determinants. Retrospective survey analysis, with 165 patients included in the study. All patients completed a survey, which included demographic data and the clinical details of their disease, the Short Form-36 Health Survey (SF-36), the revised Beck Depression Inventory (BDI-II), and the Bern Embitterment Inventory (BEI). Stepwise regression was used to identify predictors of QoL. The strongest predictors of the physical component score of the SF-36 were (in order of declining strength of association): Delay between first presentation of the disease and diagnosis, body mass index (BMI), number of doctors visited before the diagnosis of acromegaly, and age at diagnosis. For the mental component score, the strongest predictors were: number of doctors visited, previous radiotherapy, and age at study entry; and, for the BDI-II score: number of doctors visited, previous radiotherapy, age at study entry, and employment status at the time of diagnosis. The following were predictors of the BEI score: number of doctors visited, and age at study entry. Diagnostic delay and lack of diagnostic acumen in medical care provision are strong predictors of poor QoL in patients with acromegaly. Other identified parameters are radiotherapy, age, BMI, and employment status. An efficient acromegaly service should address these aspects when devising disease management plans. BDI-II = Beck Depression Inventory II BEI = Bern Embitterment Inventory BMI = body mass index IGF-1 = insulin-like growth factor 1 MCS = mental component summary (score) PCS = physical component summary (score) QoL = quality of life SDS = standard deviation score SF-36 = Short Form-36 Health Survey.

  9. [Acromegaly--from myths to facts].

    Science.gov (United States)

    Kałuzny, Marcin; Bolanowski, Marek

    2008-01-01

    The authors present numerous historical descriptions of persons who might suffered from gigantism or acromegaly. The oldest medical data of patients, initial attempts of causal neurosurgical treatment, history of growth hormone, insulin-like growth factor-1, growth hormone releasing hormone and somatostatin discovery is reported. The highest contemporary living persons are also listed.

  10. Active acromegaly enhances spontaneous parathyroid hormone pulsatility.

    Science.gov (United States)

    Mazziotti, Gherardo; Cimino, Vincenzo; De Menis, Ernesto; Bonadonna, Stefania; Bugari, Giovanna; De Marinis, Laura; Veldhuis, Johannes D; Giustina, Andrea

    2006-06-01

    In healthy subjects, parathyroid hormone (PTH) is secreted in a dual fashion, with low-amplitude and high-frequency pulses superimposed on tonic secretion. These 2 components of PTH secretion seem to have different effects on target organs. The aim of our study was to evaluate whether growth hormone excess in acromegaly may modify the spontaneous pulsatility of PTH. Five male patients with newly diagnosed active acromegaly and 8 healthy subjects were evaluated by 3-minute blood sampling for 6 hours. Plasma PTH concentrations were evaluated by multiparameter deconvolution analysis. Plasma PTH release profiles were also subjected to an approximate entropy (ApEn) estimate, which provides an ensemble measure of the serial regularity or orderliness of the release process. In acromegalic patients, baseline serum PTH values were not significantly different from those measured in the healthy subjects, as well as tonic PTH secretion rate, number of bursts, fractional pulsatile PTH secretion, and ApEn ratio. Conversely, PTH pulse half-duration was significantly longer in acromegalic patients vs healthy subjects (11.8+/-0.95 vs 6.9+/-1.6 minutes; P=.05), whereas PTH pulse mass showed a tendency (P=.06) to be significantly greater in acromegalic patients. These preliminary data suggest that growth hormone excess may affect PTH secretory dynamics in patients with acromegaly. Potentially negative bone effects of the modifications of PTH secretory pattern in acromegaly should be investigated.

  11. Lanreotide autogel in acromegaly - a decade on.

    Science.gov (United States)

    Kyriakakis, Nikolaos; Chau, Vincent; Lynch, Julie; Orme, Steve M; Murray, Robert D

    2014-12-01

    The novel formulation of lanreotide, lanreotide (LAN) autogel (ATG), has been available in Europe since 2001 and USA from 2006 for the treatment of acromegaly. It is one of only two clinically available somatostatin analogs available for use in acromegaly. Data relating to the use of ATG in acromegaly, specifically relating to comparison to octreotide (OCT) LAR and patient acceptability and preference, have been slow to accumulate. We performed a comprehensive review of the original literature relating to development, pharmacokinetics, acceptability and clinical efficacy of ATG. LAN ATG is a novel formulation of LAN consequent on self-assembly of nanotubules in water. Diffusion between molecules within the nanotubules and surrounding tissue fluid in vivo leads to pseudo first-order pharmacokinetics. Efficacy is equivalent to the alternate long-acting somatostatin analog, OCT LAR, normalizing growth hormone and IGF-I levels in around 60 and 50% respectively. Control of tumor growth is observed in over 95% of patients, with 64% seeing a clinically significant reduction in tumor size. ATG is provided in a prefilled syringe for deep subcutaneous injection, allowing self-injection, and may be administered up to 8 weeks greatly improving convenience for the patient. The data strongly support consideration of ATG as the medical therapy of choice for patients with acromegaly.

  12. Gigantism and Acromegaly Due to Xq26 Microduplications and GPR101 Mutation

    Science.gov (United States)

    Trivellin, G.; Daly, A.F.; Faucz, F.R.; Yuan, B.; Rostomyan, L.; Larco, D.O.; Schernthaner-Reiter, M.H.; Szarek, E.; Leal, L.F.; Caberg, J.-H.; Castermans, E.; Villa, C.; Dimopoulos, A.; Chittiboina, P.; Xekouki, P.; Shah, N.; Metzger, D.; Lysy, P.A.; Ferrante, E.; Strebkova, N.; Mazerkina, N.; Zatelli, M.C.; Lodish, M.; Horvath, A.; de Alexandre, R. Bertollo; Manning, A.D.; Levy, I.; Keil, M.F.; de la Luz Sierra, M.; Palmeira, L.; Coppieters, W.; Georges, M.; Naves, L.A.; Jamar, M.; Bours, V.; Wu, T.J.; Choong, C.S.; Bertherat, J.; Chanson, P.; Kamenický, P.; Farrell, W.E.; Barlier, A.; Quezado, M.; Bjelobaba, I.; Stojilkovic, S.S.; Wess, J.; Costanzi, S.; Liu, P.; Lupski, J.R.; Beckers, A.; Stratakis, C.A.

    2015-01-01

    BACKGROUND Increased secretion of growth hormone leads to gigantism in children and acromegaly in adults; the genetic causes of gigantism and acromegaly are poorly understood. METHODS We performed clinical and genetic studies of samples obtained from 43 patients with gigantism and then sequenced an implicated gene in samples from 248 patients with acromegaly. RESULTS We observed microduplication on chromosome Xq26.3 in samples from 13 patients with gigantism; of these samples, 4 were obtained from members of two unrelated kindreds, and 9 were from patients with sporadic cases. All the patients had disease onset during early childhood. Of the patients with gigantism who did not carry an Xq26.3 microduplication, none presented before the age of 5 years. Genomic characterization of the Xq26.3 region suggests that the microduplications are generated during chromosome replication and that they contain four protein-coding genes. Only one of these genes, GPR101, which encodes a G-protein–coupled receptor, was overexpressed in patients’ pituitary lesions. We identified a recurrent GPR101 mutation (p.E308D) in 11 of 248 patients with acromegaly, with the mutation found mostly in tumors. When the mutation was transfected into rat GH3 cells, it led to increased release of growth hormone and proliferation of growth hormone–producing cells. CONCLUSIONS We describe a pediatric disorder (which we have termed X-linked acrogigantism [X-LAG]) that is caused by an Xq26.3 genomic duplication and is characterized by early-onset gigantism resulting from an excess of growth hormone. Duplication of GPR101 probably causes X-LAG. We also found a recurrent mutation in GPR101 in some adults with acromegaly. (Funded by the Eunice Kennedy Shriver National Institute of Child Health and Human Development and others.) PMID:25470569

  13. Gigantism and acromegaly due to Xq26 microduplications and GPR101 mutation.

    Science.gov (United States)

    Trivellin, Giampaolo; Daly, Adrian F; Faucz, Fabio R; Yuan, Bo; Rostomyan, Liliya; Larco, Darwin O; Schernthaner-Reiter, Marie Helene; Szarek, Eva; Leal, Letícia F; Caberg, Jean-Hubert; Castermans, Emilie; Villa, Chiara; Dimopoulos, Aggeliki; Chittiboina, Prashant; Xekouki, Paraskevi; Shah, Nalini; Metzger, Daniel; Lysy, Philippe A; Ferrante, Emanuele; Strebkova, Natalia; Mazerkina, Nadia; Zatelli, Maria Chiara; Lodish, Maya; Horvath, Anelia; de Alexandre, Rodrigo Bertollo; Manning, Allison D; Levy, Isaac; Keil, Margaret F; Sierra, Maria de la Luz; Palmeira, Leonor; Coppieters, Wouter; Georges, Michel; Naves, Luciana A; Jamar, Mauricette; Bours, Vincent; Wu, T John; Choong, Catherine S; Bertherat, Jerome; Chanson, Philippe; Kamenický, Peter; Farrell, William E; Barlier, Anne; Quezado, Martha; Bjelobaba, Ivana; Stojilkovic, Stanko S; Wess, Jurgen; Costanzi, Stefano; Liu, Pengfei; Lupski, James R; Beckers, Albert; Stratakis, Constantine A

    2014-12-18

    Increased secretion of growth hormone leads to gigantism in children and acromegaly in adults; the genetic causes of gigantism and acromegaly are poorly understood. We performed clinical and genetic studies of samples obtained from 43 patients with gigantism and then sequenced an implicated gene in samples from 248 patients with acromegaly. We observed microduplication on chromosome Xq26.3 in samples from 13 patients with gigantism; of these samples, 4 were obtained from members of two unrelated kindreds, and 9 were from patients with sporadic cases. All the patients had disease onset during early childhood. Of the patients with gigantism who did not carry an Xq26.3 microduplication, none presented before the age of 5 years. Genomic characterization of the Xq26.3 region suggests that the microduplications are generated during chromosome replication and that they contain four protein-coding genes. Only one of these genes, GPR101, which encodes a G-protein-coupled receptor, was overexpressed in patients' pituitary lesions. We identified a recurrent GPR101 mutation (p.E308D) in 11 of 248 patients with acromegaly, with the mutation found mostly in tumors. When the mutation was transfected into rat GH3 cells, it led to increased release of growth hormone and proliferation of growth hormone-producing cells. We describe a pediatric disorder (which we have termed X-linked acrogigantism [X-LAG]) that is caused by an Xq26.3 genomic duplication and is characterized by early-onset gigantism resulting from an excess of growth hormone. Duplication of GPR101 probably causes X-LAG. We also found a recurrent mutation in GPR101 in some adults with acromegaly. (Funded by the Eunice Kennedy Shriver National Institute of Child Health and Human Development and others.).

  14. Muscle strength in patients with acromegaly at diagnosis and during long-term follow-up.

    Science.gov (United States)

    Füchtbauer, Laila; Olsson, Daniel S; Bengtsson, Bengt-Åke; Norrman, Lise-Lott; Sunnerhagen, Katharina S; Johannsson, Gudmundur

    2017-08-01

    Patients with acromegaly have decreased body fat (BF) and increased extracellular water (ECW) and muscle mass. Although there is a lack of systematic studies on muscle function, it is believed that patients with acromegaly may suffer from proximal muscle weakness despite their increased muscle mass. We studied body composition and muscle function in untreated acromegaly and after biochemical remission. Prospective observational study. Patients with acromegaly underwent measurements of muscle strength (dynamometers) and body composition (four-compartment model) at diagnosis (n = 48), 1 year after surgery (n = 29) and after long-term follow-up (median 11 years) (n = 24). Results were compared to healthy subjects. Untreated patients had increased body cell mass (113 ± 9% of predicted) and ECW (110 ± 20%) and decreased BF (67 ± 7.6%). At one-year follow-up, serum concentration of IGF-I was reduced and body composition had normalized. At baseline, isometric muscle strength in knee flexors and extensors was normal and concentric strength was modestly increased whereas grip strength and endurance was reduced. After one year, muscle strength was normal in both patients with still active disease and patients in remission. At long-term follow-up, all patients were in remission. Most muscle function tests remained normal, but isometric flexion and the fatigue index were increased to 153 ± 42% and 139 ± 28% of predicted values, respectively. Patients with untreated acromegaly had increased body cell mass and normal or modestly increased proximal muscle strength, whereas their grip strength was reduced. After biochemical improvement and remission, body composition was normalized, hand grip strength was increased, whereas proximal muscle fatigue increased. © 2017 European Society of Endocrinology.

  15. Adolescent acromegaly: clinical parameters and treatment outcome.

    Science.gov (United States)

    Bhansali, A; Upreti, V; Dutta, P; Mukherjee, K K; Nahar, U; Santosh, R; Das, S; Walia, R; Pathak, A

    2010-10-01

    Adolescent acromegaly is a rare disorder and these patients present with tall stature/gigantism, tumor mass effects and menstrual irregularities. 34 consecutive (26 males) patients having onset of disease prior to 21 years of age were included in this retrospective analysis. Their clinical features and treatment outcome were studied. Mean age and lag time at presentation were 21.6 +/- 3.9 years and 5.1 +/- 3.5 years respectively. Common presenting manifestations included acral enlargement, tumor mass effects and menstrual irregularities. Mean height at presentation was 174.6 +/- 13.7 cms (range: 150-210 cm) and one third had gigantism (height > or =97th percentile, WHO growth charts). Hypertension and glucose intolerance were seen in 15% and 23.5% respectively. Mean nadir GH after glucose load was 58.2 +/- 13.7 ng/ml and IGF -1 was 534.8 +/- 132.8 ng/ml. Half of the patients had concomitant hyperprolactinemia. Almost all (97%) had macroadenoma and anterior pituitary hormone deficiencies were frequent (75%). Patients with gigantism were younger (19.6 +/- 4.9 vs. 22.6 +/- 2.9 years; p = 0.001), had higher GH values (66.68 +/- 27.22 vs. 53.98 +/- 15.99 ng/ml; p = 0.04) and hypogonadism was more common (90.9% vs. 56.5%, p = 0.03) than those with normal stature. 32 patients (94.1%) were treated primarily with surgery, 7 (21.9%) received post operative radiotherapy. Mean duration of follow up was 33.1 +/- 10.1 months. Only 30% had nadir GH values of <1 ng/ml. One third of adolescent patients had acrogigantism. These patients were younger, had higher GH levels and concurrent hypogonadism was more common. Cure could be achieved only in about one third of the patients.

  16. Elastosonographic evaluation of thyroid nodules in acromegaly.

    Science.gov (United States)

    Scacchi, Massimo; Andrioli, Massimiliano; Carzaniga, Chiara; Vitale, Giovanni; Moro, Mirella; Poggi, Luca; Pecori Giraldi, Francesca; Fatti, Letizia M; Cavagnini, Francesco

    2009-10-01

    Ultrasound-elastography (US-E) appears to be a helpful tool for the diagnosis of thyroid cancer. In acromegaly, the prevalence of thyroid cancer is still debated. The aims of this study were to evaluate thyroid nodules in acromegaly and to establish the accuracy of US-E in providing information on their nature, using cytological analysis as a reference. US-E was applied to 90 nodules detected in 25 acromegalic patients and to 94 nodules found in 31 non-acromegalic goitrous subjects. The lesions were classified according to the elasticity scores (ES) as soft (ES 1-2) or hard (ES 3-4). Fine needle aspiration cytology could be performed in 60.8% of hard nodules in acromegalics and in 86.7% of hard nodules in controls. The prevalence of hard nodules was significantly higher in the whole group of acromegalic patients than in controls (56.8 vs 16.0%, P<0.0001). The prevalence of hard nodules in patients with active acromegaly (68.9%) was greater, though not to a statistically significant extent, than that observed in cured (44.4%) and controlled (52.5%) patients. Cytology revealed malignancy or suspect malignancy in four of the nodules of non-acromegalic subjects and in none of the nodules of acromegalic patients. This study has demonstrated a high prevalence of stiff thyroid nodules in acromegaly, greater than that found in non-acromegalic goitrous subjects. In acromegalics, hard nodules appeared not to be malignant on cytopathological examination and are probably of fibrous nature. Thus, US-E appears to be of limited value for the diagnosis of thyroid cancer in acromegaly.

  17. Adiponectin and Cardiac Hypertrophy in Acromegaly.

    Science.gov (United States)

    Gurbulak, Sabriye; Akin, Fulya; Yerlikaya, Emrah; Yaylali, Guzin F; Topsakal, Senay; Tanriverdi, Halil; Akdag, Beyza; Kaptanoglu, Bunyamin

    2016-01-01

    Adiponectin is an adipocytes-derived hormone which has been shown to possess insulin-sensitizing, antiatherogenic, and anti-inflammatory properties. In acromegaly, the data on adiponectin is contradictory. The relationship between adiponectin levels and cardiac parameters has not been studied. The aim of this study was to find out how adiponectin levels were affected in acromegalic patients and the relationship between adiponectin levels and cardiac parameters. We included 30 subjects (15 male, 15 female), diagnosed with acromegaly and 30 healthy (10 male, 20 female) subjects. Serum glucose, insulin, GH, IGF-1 and adiponectin levels were obtained and the insulin resistance of the subjects was calculated. Echocardiographic studies of the subjects were performed. We determined that adiponectin levels were significantly higher in the acromegalic group than the control group. In the acromegalic group, there was no statistically significant relation between serum adiponectin and growth hormone (GH), or insulin-like growth factor-1 (IGF-1) levels (p = 0.3, p = 0.1). We demonstrated that cardiac function and structure are affected by acromegaly. IVST, PWT, LVMI, E/A ratio, DT, ET, IVRT, VPR, and LVESV values were increased and the results were statistically significant. In the acromegalic group, adiponectin levels were positively related with left ventricle mass index (LVMI) but this correlation was found to be statistically weak (p = 0.03). In our study, there was a positive correlation between VAI and LVM. We also could not find any correlation between VAI and adiponectin levels. Although insulin resistance and high insulin levels occur in active acromegaly patients, adiponectin levels were higher in our study as a consequence of GH lowering therapies. Our study showed that adiponectin levels may be an indicator of the cardiac involvement acromegaly. However, the usage of serum adiponectin levels in acromegalic patients as an indicator of cardiac involvement should be

  18. Growth hormone modulation of arginine-induced glucagon release: studies of isolated growth hormone deficiency and acromegaly.

    Science.gov (United States)

    Seino, Y; Taminato, T; Goto, Y; Inoue, Y; Kadowaki, S; Hattori, M; Mori, K; Kato, Y; Matsukura, S; Imura, H

    1978-12-01

    Plasma glucagon and insulin responses to L-arginine were compared in normal controls and patients with isolated growth hormone deficiency and acromegaly. Patients with isolated growth hormone deficiency were characterized by high plasma glucagon response and low plasma insulin response, whereas acromegalic patients showed exaggerated plasma glucagon response and almost normal insulin response. These results suggest that growth hormone is probably required for optimum function of the islets, and since hyperglucagonaemia was observed in both growth hormone deficiency and acromegaly, metabolic disturbances stemming from the respective primary diseases may affect glucagon secretion.

  19. Acromegaly and aging: a comparative cross-sectional study.

    Science.gov (United States)

    Hatipoglu, Esra; Yuruyen, Mehmet; Keskin, Ela; Yavuzer, Hakan; Niyazoglu, Mutlu; Doventas, Alper; Erdincler, Deniz Suna; Beger, Tanju; Kadioglu, Pinar; Gundogdu, Sadi

    2015-02-01

    Cognitive and functional geriatric assessment may change in acromegaly. Herein we aimed to determine at which points geriatric assessment of the cases with acromegaly differs from that of general elderly population. In this comparative cross-sectional study, a total of 30 cases with acromegaly (controlled n = 14, uncontrolled n = 16) and 30 gender and body-mass index-matched cases without acromegaly (control group, CG) above 60 years old were included. Cognitive functions were evaluated on the basis of the mini-mental state exam (MMSE). Affective status was determined using the geriatric depression scale. Activities of daily living (ADL) were ranked according to the Barthel index while instrumental activities of daily living (IADL) were graded on the basis of the Lawton scale. Nutritional status was evaluated using the mini-nutritional assessment (MNA). Body composition was measured through bioimpedance analysis. Functional mobility was determined using the Timed Up and Go test (TUG) and muscle strength with the handgrip strength test. Scores on the MMSE were significantly lower in the elderly cases with acromegaly than in the cases without acromegaly (p acromegaly group than in the CG (p = 0.04). Total MNA scores were significantly lower in cases with acromegaly than in the CG (p = 0.006). More subjects in the acromegaly group (33%) were at greater risk of malnutrition than in the CG (3%) (p = 0.003). There was greater moderate functional impairment based on Barthel ADL in the acromegaly group than in the CG (p = 0.04). Acromegaly may impair cognitive functions, functional mobility and instrumental daily living activities in the geriatric population. With acromegaly, the risk of malnutrition may also increase. Copyright © 2014 Elsevier Ltd. All rights reserved.

  20. [The medical management of Cushing's syndrome].

    Science.gov (United States)

    Leal-Cerro, Alfonso; Soto Moreno, Alfonso; Mangas, Miguel Angel; León Justel, Antonio; Webb, Susan

    2009-04-01

    Cushing's syndrome results from prolonged exposure to excessive circulating glucocorticosteroids and is associated with significant morbidity and mortality. While the treatment of choice in most patients is surgical, the metabolic consequences of this syndrome, including hypertension and diabetes mellitus, increase the risks of such surgery. Hypercortisolemia and its sequelae can be efficiently reversed or controlled using medical therapy, either as a temporary measure prior to definitive treatment or as a longer-term treatment in some particularly difficult cases. Drug treatment has been targeted at the hypothalamic/pituitary level, the adrenal glands and at glucocorticoid receptors. The present review discusses the pharmacotherapeutic agents that have been used in Cushing's syndrome and the criteria for their use, as well as recent drugs that may improve the medical treatment of this complex endocrinological disorder in the future. Finally, the short-and long-term follow-up of patients with Cushing's syndrome after surgery is also discussed.

  1. More concerns and stronger beliefs about the necessity of medication in patients with acromegaly are associated with negative illness perceptions and impairment in quality of life.

    Science.gov (United States)

    Andela, Cornelie D; Biermasz, Nienke R; Kaptein, Adrian A; Pereira, Alberto M; Tiemensma, Jitske

    2015-10-01

    Patients with acromegaly can be treated with surgery, radiotherapy and/or medical treatment. In general, patients' beliefs about medication are associated with illness perceptions, a contributory factor of Quality of Life (QoL). At present, there are no quantitative studies on medication beliefs in patients with acromegaly. Here, we aimed to examine possible associations between medication beliefs, illness perceptions, and QoL. Furthermore we aimed to explore whether illness perceptions of patients with remission of acromegaly receiving medical treatment differ from patients without medical treatment. Cross-sectional evaluation of 73 patients with remission of acromegaly (n = 28 patients with medication, n = 45 without medication). The Beliefs about Medicines Questionnaire (BMQ), Illness Perception Questionnaire-Revised (IPQ-R), EuroQoL-5D, and AcroQoL were used for the assessment. Stronger beliefs about the necessity of medical treatment and stronger concerns about the adverse effects were associated with attributing more symptoms to acromegaly, perceiving more negative consequences, and having a stronger belief in a cyclical timeline (BMQ, all P acromegaly tend to perceive a more chronic timeline of their disease, compared to patients with remission without medical treatment. These psychological factors need to be taken into account when treating patients and developing a psychosocial education program aiming to improve QoL. Copyright © 2015 Elsevier Ltd. All rights reserved.

  2. A dangerous Cushing response in a child: neurogenic heart damage.

    Science.gov (United States)

    Ruggieri, Francesco; Calvi, Maria Rosa; Beretta, Luigi

    2014-04-01

    Cushing response, which acts to preserve cerebral blood flow by inducing arterial hypertension, could induce neurogenic heart damage through hyperactivation of autonomic nervous system. Most of clinical reports describe neurogenic heart damage as a self-limiting condition clinically characterized by electrocardiographic abnormalities in the setting of an acute neurologic insult. Here we describe a case of life-threatening cardiac dysfunction immediately after a massive intracerebral hemorrhage in a healthy 7-year-old child. The low probability of ischemic heart disease, the poor increase of cardiac necrosis markers, the localization of regional wall motion abnormalities that are not typical for coronary artery disease, and reversibility after brain surgical decompression are consistent all with neurogenic heart damage. Acute decrease of brain oxygen delivery caused by cardiac dysfunction worsens secondary brain injury in the setting of an acute neurologic insult. Thus, Cushing response, which is a physiological mechanism of cerebral protection, could become a double-edged sword when massive sympathetic activation makes the myocardium stunned.

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

    Science.gov (United States)

    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 educational nursing program improved 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.

  4. Reduction of elevated IGF-1 levels in coincident amyotrophic lateral sclerosis and acromegaly.

    Science.gov (United States)

    Pereira, Erlick A C; Turner, Martin R; Wass, John A H; Talbot, Kevin

    2010-01-01

    We report a patient presenting with ALS in whom acromegaly was later confirmed. Insulin-like growth factor-1 (IGF-1) has been tried in the treatment of ALS and despite equivocal results from clinical trials, efforts have continued to try to harness the significant positive effects on motor neuron growth observed in vitro and in survival of mouse models of the disease. One subsequent study has reported an association between higher circulating serum IGF-1 levels and longer disease duration in ALS patients. Concern therefore arose in our case that treatment of the acromegaly with a somatostatin analogue might adversely affect the natural course of his ALS through lowering of potentially beneficial IGF-1 levels. Through clinical observation and prognostic modelling we suggest that this concern was unfounded. The potential interaction of these two rarely coincident disorders in our patient is discussed.

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

    Science.gov (United States)

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

    2013-06-01

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

  6. Value of [18F] fluoro-2-deoxy-D-glucose Positron Emission Tomography/Computed Tomography in Diagnosis and Localization of Cushing's Disease%18F-脱氧葡萄糖正电子发射计算机断层显像在库欣病诊断和术前定位中的价值

    Institute of Scientific and Technical Information of China (English)

    程欣; 崔瑞雪; 潘慧; 袁涛; 朱惠娟; 李方

    2011-01-01

    目的 评价18F-脱氧葡萄糖(FDG)正电子发射计算机断层显像 (PET)/CT在库欣病定性和定位诊断中的价值.方法 12 例经口鼻蝶窦垂体腺瘤切除后病理证实为库欣病患者,术前行 FDG PET/CT躯干和脑显像,同期行鞍区核磁共振成像 (MRI)和奥曲肽全身显像,6例行岩下窦静脉取血 (IPSS).结果 12 例PET/CT 躯干显像均未见异常,脑显像对垂体病变诊断的阳性率为91.6%(11/12),MRI对垂体病变诊断的阳性率为66.7%(8/12),6例IPSS 中5例定位为垂体,定侧准确率为50%(3/6).结论 FDG PET/CT躯干显像可协助除外异位促肾上腺皮质激素综合征,而脑显像对库欣病定位的准确率明显高于MRI,尤其对MRI检查阴性和IPSS无法定位患者的术前诊断有重要意义.%Objective To explore the value of [ 18F ] fluoro-2-deoxy-D-glucose ( 18 FDG) positron emission tomography and computer tomography (PET/CT) in the qualitative diagnosis and localization of Cushing's disease. Methods Totally 12 patients underwent transsphenoidal adenomeetomy and were histopathologieally proven to be with Cushing's disease. 18FDG PET/CT whole-body and brain scannings were performed preoperatively; meanwhile, magnetic resonance imaging (MRI) and 99mTc-octreotide examination were done in all 12 cases, and inferior petrosal sinus sampling (IPSS) were done in 6 patients. Results The sensitivity of 18FDG in diagnosing Cushing's disease was 91.6% (11/12) , but URI was 66.7% (8/12). For the 6 patients who performed IPSS, 5 of them was diagnosed to be with Cushing's disease, and only 50% (3/6)were localized correctly in the pituitary gland. Conclusions 18FDG PET/CT whole-body scan can exclude ectopic adrenocorticotropin-secreting tumors, and localize the pituitary lesions with higher accuracy than MRI.Therefore, it is useful for suspected Cushing's disease, especially for patients their MRI and IPSS have negative or paradoxical results.

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

    DEFF Research Database (Denmark)

    Andreassen, Mikkel; Faber, Jens Oscar; 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...

  8. Hypertrophic remodeling of subcutaneous small resistance arteries in patients with Cushing's syndrome.

    Science.gov (United States)

    Rizzoni, Damiano; Porteri, Enzo; De Ciuceis, Carolina; Rodella, Luigi F; Paiardi, Silvia; Rizzardi, Nicola; Platto, Caterina; Boari, Gianluca E M; Pilu, Annamaria; Tiberio, Guido A M; Giulini, Stefano M; Favero, Gaia; Rezzani, Rita; Rosei, Claudia Agabiti; Bulgari, Giuseppe; Avanzi, Daniele; Rosei, Enrico Agabiti

    2009-12-01

    Structural alterations of small resistance arteries in essential hypertensive patients (EH) are mostly characterized by inward eutrophic remodeling. However, we observed hypertrophic remodeling in patients with renovascular hypertension, in those with acromegaly, as well as in patients with non-insulin-dependent diabetes mellitus, suggesting a relevant effect of humoral growth factors on vascular structure, even independent from the hemodynamic load. Cortisol may stimulate the renin-angiotensin system and may induce cardiac hypertrophy. However, presently no data are available about small artery structure in patients with Cushing's syndrome. We have investigated the structure of sc small resistance arteries in 12 normotensive subjects (NT), in 12 EH subjects, and in eight patients with Cushing's syndrome (CS). Small arteries from sc fat were dissected and mounted on a micromyograph. The normalized internal diameter, media thickness, media to lumen ratio, and the media cross-sectional area were measured, as well as indices of oxidative stress. Demographic variables were similar in the three groups, except for clinic blood pressure. The media to lumen ratio was significantly greater in EH and CS, compared with NT; no difference was observed between EH and CS. The media cross-sectional area was significantly greater in CS compared with EH and with NT. An increased vascular oxidative stress was present in CS, as demonstrated by increased levels of superoxide anions, cyclooxygenase-1 and endothelial nitric oxide synthase in the microvessels. Our results suggest the presence of hypertrophic remodeling in sc small resistance arteries of CS, probably as a consequence of growth-promoting properties of circulating cortisol and/or increased vascular oxidative stress.

  9. Binswanger's Disease

    Science.gov (United States)

    ... Craniosynostosis Information Page Creutzfeldt-Jakob Disease Information Page Cushing's Syndrome Information Page Dandy-Walker Syndrome Information Page Deep Brain Stimulation for Parkinson's Disease Information Page Dementia Information ...

  10. Batten Disease

    Science.gov (United States)

    ... Craniosynostosis Information Page Creutzfeldt-Jakob Disease Information Page Cushing's Syndrome Information Page Dandy-Walker Syndrome Information Page Deep Brain Stimulation for Parkinson's Disease Information Page Dementia Information ...

  11. Behcet's Disease

    Science.gov (United States)

    ... Craniosynostosis Information Page Creutzfeldt-Jakob Disease Information Page Cushing's Syndrome Information Page Dandy-Walker Syndrome Information Page Deep Brain Stimulation for Parkinson's Disease Information Page Dementia Information ...

  12. Krabbe Disease

    Science.gov (United States)

    ... Craniosynostosis Information Page Creutzfeldt-Jakob Disease Information Page Cushing's Syndrome Information Page Dandy-Walker Syndrome Information Page Deep Brain Stimulation for Parkinson's Disease Information Page Dementia Information ...

  13. Thyroid lesions in patients with acromegaly - case-control study and update to the meta-analysis.

    Science.gov (United States)

    Woliński, Kosma; Stangierski, Adam; Gurgul, Edyta; Bromińska, Barbara; Czarnywojtek, Agata; Lodyga, Martha; Ruchała, Marek

    2017-01-01

    Acromegaly results from oversecretion of growth hormone and subsequently insulin growth factor-1. According to some authors, the disease can cause increased prevalence of nodular goitre and thyroid cancer (TC). However, the number of studies comparing acromegalic patients with control groups is low. We aimed to assess the prevalence of thyroid lesions in patients with acromegaly in comparison to an age- and sex-matched control group and to update the meta-analysis previously performed in our department by the same authors. We searched medical documentation of patients with acromegaly treated in our department between 2003 and 2013. The prevalence of thyroid abnormalities was compared with the group of patients with hormonally inactive adrenal incidentalomas. To perform the meta-analytic part of the paper we also searched ten databases to find relevant papers. Two hundred and five patients with acromegaly and 184 patients with incidentalomas were included. Any thyroid lesions were present in 77.6% of patients with acromegaly vs. 63.0% with incidentalomas (p = 0.002), multinodular goitre - 66.8% vs. 47.8% (p = 0.0002), and TC- 5.4% vs. 2.7% (p = 0.21) respectively. For thyroid lesions the pooled odds ratio (OR) was 3.1 (95% confidence interval [CI] 1.8-5.5), and for TCs the OR was 4.5 (95% CI 1.9-10.3). According to our results thyroid lesions were significantly more common in patients with acromegaly; in case of TC the difference was not significant. The updated meta-analysis showed significantly increased prevalence of both disorders. In conclusion, systematic thyroid examination should be an important part of follow-up in case of acromegalic patients. (Endokrynol Pol 2017; 68 (1): 2-6).

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

    DEFF Research Database (Denmark)

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

    2001-01-01

    The aim was to evaluate, markers of disease activity in acromegaly in relation to perceived disease activity. Thirty-seven consecutively treated, acromegalic patients, classified by clinical symptoms as inactive (n=16), slightly active (n=10) and active (n=11), entered the study. When evaluating......-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...

  15. Recent advances in understanding Cushing disease: resistance to glucocorticoid negative feedback and somatic USP8 mutations [version 1; referees: 2 approved

    Directory of Open Access Journals (Sweden)

    Eleni Daniel

    2017-05-01

    Full Text Available Cushing’s disease is a rare disease with a characteristic phenotype due to significant hypercortisolism driven by over-secretion of adrenocorticotropic hormone and to high morbidity and mortality if untreated. It is caused by a corticotroph adenoma of the pituitary, but the exact mechanisms leading to tumorigenesis are not clear. Recent advances in molecular biology such as the discovery of somatic mutations of the ubiquitin-specific peptidase 8 (USP8 gene allow new insights into the pathogenesis, which could be translated into exciting and much-needed therapeutic applications.

  16. Cushing综合征33例回顾分析%Clinical Analysis of 33 Cases with Cushing's Syndrome

    Institute of Scientific and Technical Information of China (English)

    王浩

    2013-01-01

    目的回顾分析Cushing综合征的病因、临床表现、诊断和治疗方法。方法收集我院近10年的Cushing综合征患者资料,并进行回顾性分析。结果在本组病例中,Cushing综合征患者多表现为中心性肥胖、皮肤紫纹、高血压、低血钾等。病因诊断ACTH瘤占54.5%,肾上腺腺瘤占30%。治疗上多采用手术。结论ACTH瘤和肾上腺肿瘤是Cushing综合征最重要的病因。实验室检查中,除ATCH、Cor异常外,低血钾、血糖异常多见。%Objective To analyse the clinical and laboratory characteristics of Cushing's syndrome. Methods The clinical data of 33 cases with Cushing's syndrome from recent years were col ected and analysed. Results The characteristics of these patients include central obesity,hypertension and hypokalemia . Among the etiology ,Cushing disease and adrenal ademoma are 54.5% and 30%,respectively.The thepary involve surgery. Conclusion Cushing disease and adrenal ademoma are the most common cause of Cushing syndrom. Hypocalemia and glucose abnormal are often happen.

  17. Lung carcinoid related Cushing's syndrome%肺类癌相关性库欣综合征

    Institute of Scientific and Technical Information of China (English)

    饶颖; 郑少雄

    2009-01-01

    肺类癌相关性库欣综合征为一罕见疾病,本文简要介绍了4例患者的临床体征、治疗等情况,以期对临床医生有所帮助.%Lung carcinoid related Cushing's syndrome(LCRCS)is a rare disease. This article sum-marizes the symptoms and treatments of four patients with LCRCS and helps the clinicians to distinguish this kind of disease from Cushing's syndrome.

  18. Acromegaly according to the Danish National Registry of Patients: how valid are ICD diagnoses and how do patterns of registration affect the accuracy of registry data?

    Directory of Open Access Journals (Sweden)

    Dal J

    2014-09-01

    Full Text Available Jakob Dal,1 Nikolaj Skou,1 Eigil Husted Nielsen,2 Jens Otto Lunde Jørgensen,1 Lars Pedersen3 1Department of Endocrinology, Aarhus University Hospital, Aarhus, 2Department of Endocrinology, Aalborg University Hospital, Aalborg, 3Department of Clinical Epidemiology, Aarhus University Hospital, Aarhus, Denmark Background: The incidence of acromegaly is uncertain, since population-based studies are few. In the absence of a specific acromegaly registry, the Danish National Registry of Patients (DNRP becomes a potential source of data for studying the epidemiology of acromegaly, by linking all hospital discharge diagnoses to the personal identification numbers of individual Danish inhabitants. The validity of the DNRP with respect to acromegaly, however, remains to be tested. The aim of this study was to validate the International Classification of Diseases (ICD codes for acromegaly (ICD-8: 25300, 25301. ICD-10: E22.0 as used in the DNRP, and to assess the influence of various registration patterns on the accuracy of registry data. Methods: We identified patients registered with ICD codes for the diagnosis of acromegaly or other pituitary disorders during the period 1991–2009. Data on the institutional origin of each registration and the number of relevant DNRP registrations were recorded, and systematic patient chart reviews were performed to confirm the diagnosis. Results: In total, 110 cases of acromegaly were confirmed, compared with 275 registered cases, yielding a positive predictive value (PPV of 40%. When restricting the search to the regional highly specialized department of endocrinology, the PPV increased to 53% with no loss of cases with confirmed acromegaly. With a requirement of at least one, two, or three DNRP registrations, the PPV increased, but with a concurrent loss of confirmed cases. Conclusion: The DNRP seems to be a useful source for identifying new cases of acromegaly, especially when restricting the search to a relevant

  19. A rare cause of Cushing's syndrome

    DEFF Research Database (Denmark)

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

    2014-01-01

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

  20. Aberrations in carbohydrate metabolism in patients with diagnosed acromegaly, hospitalized in the Endocrinology and Diabetology Department of Collegium Medicum University of Nicolaus Copernicus in Bydgoszcz in the years 2001-2009.

    Science.gov (United States)

    Gierach, Marcin; Gierach, Joanna; Pujanek, Małgorzata; Skowrońska, Agnieszka; Rutkowska, Emilia; Junik, Roman

    2010-01-01

    The incidence rate of acromegaly is 50 to70 persons per one million people. It occurs twice as often in women than in men, especially in middle age. In the course of the disease, insulin resistance develops, which is connected with the hypersecretion of somatotropin. Type 2 diabetes mellitus occurs more frequently in patients with acromegaly than in the rest of the population. It is diagnosed in 13-56% of acromegaly sufferers; impaired glucose tolerance occurs in 16-46% of examined patients with acromegaly. The aim of this study is to estimate disturbances of glycaemia in patients with diagnosed acromegaly, hospitalized in the Endocrinology and Diabetology Department of Collegium Medicum University of Nicolaus Copernicus in Bydgoszcz in the years 2001-2009. The participants were a group of 70 patients with acromegaly diagnosed on the basis of clinical symptoms, high levels of somatotropin, and MRI imaging of the pituitary gland. Type 2 diabetes was diagnosed in 19 patients (27%); there were 11 cases (15%) of diagnosis of impaired fasting glycaemia (IFG) and imapired glucose tolerance (IGT) combined. Normoglycaemia was recognized in 57% of participants (40 patients). The average age of participants with concomitant type 2 diabetes was 56.1 years, whereas the average age of those without carbohydrate metabolism disturbances was 47.1 years. In patients with acromegaly, the incidence rate of type 2 diabetes is 3-4-times higher than in the rest of the population and increases with age, especially after the age of 60.

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

    Science.gov (United States)

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

    2017-08-01

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

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

    Science.gov (United States)

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

    2016-01-01

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

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

    Science.gov (United States)

    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.

  4. MRI of pituitary adenomas in acromegaly

    Energy Technology Data Exchange (ETDEWEB)

    Marro, B. [Service de Neuroradiologie, Batiment Babinsky, Hopital Salpetriere, 47, Bd de l`Hopital, F-75013 Paris (France); Zouaoui, A. [Service de Neuroradiologie, Batiment Babinsky, Hopital Salpetriere, 47, Bd de l`Hopital, F-75013 Paris (France); Sahel, M. [Service de Neuroradiologie, Batiment Babinsky, Hopital Salpetriere, 47, Bd de l`Hopital, F-75013 Paris (France); Crozat, N. [Service de Neuroradiologie, Batiment Babinsky, Hopital Salpetriere, 47, Bd de l`Hopital, F-75013 Paris (France); Gerber, S. [Service de Neuroradiologie, Batiment Babinsky, Hopital Salpetriere, 47, Bd de l`Hopital, F-75013 Paris (France); Sourour, N. [Service de Neuroradiologie, Batiment Babinsky, Hopital Salpetriere, 47, Bd de l`Hopital, F-75013 Paris (France); Sag, K. [Service de Neuroradiologie, Batiment Babinsky, Hopital Salpetriere, 47, Bd de l`Hopital, F-75013 Paris (France); Marsault, C. [Service de Neuroradiologie, Batiment Babinsky, Hopital Salpetriere, 47, Bd de l`Hopital, F-75013 Paris (France)

    1997-06-01

    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.

  5. Treatment of acromegaly by external irradiation

    Energy Technology Data Exchange (ETDEWEB)

    Macleod, A.F.; Clarke, D.G.; Pambakian, H.; Lowy, C.; Sonksen, P.H.; Collins, C.D. (Saint Thomas' Hospital, London (UK))

    1989-03-01

    Despite hypophysectomy as the primary treatment in acromegaly, external radiotherapy maintains a role as a relatively slow but effective therapy for inadequately treated patients or those unsuitable for operation. Over the last 25 years the authors' regimen has differed from published series in that they give a larger dose per fraction, with fewer treatments. They have analysed the efficacy and side-effects of this regimen in 27 subjects with acromegaly. Growth hormone levels have fallen by, on average, 27% per year in the first five years, 83% of subjects achieving a basal growth hormone of <10mU/1. The acute and chronic side-effects of irradiation are discussed, including the relevance of estimates of biological potency, for example Time Dose Fraction (TDF). One patient suffered visual loss that was most likely to be secondary to the radiotherapy. Histological appearances of the pituitary fossa in five subjects previously treated with radiotherapy are reported. (author).

  6. Ruptured chordae tendineae in acromegaly. An autopsy case.

    Science.gov (United States)

    Kaku, T; Nakashima, Y; Ichiyasu, H; Soejima, M; Baba, K; Kuroiwa, A

    1991-07-01

    A 57-year-old woman with acromegaly associated with mitral chordal rupture is reported. She was noted to have abnormal development in the size of her hands and feet in childhood. She occasionally suffered from shortness of breath on exertion and nocturnal dyspnea for several years, and was diagnosed and treated as having congestive heart failure due to valvular heart disease. On admission to our hospital, chordal rupture was suspected on the basis of M-mode and two-dimensional echocardiography. Seven years after discharge, she died of congestive heart failure. On autopsy, the heart weight and ventricular wall thickness were increased. Rupture of the posterior chordae was confirmed, but evidence of an old myocardial infarction was not found. There was severe interstitial fibrosis in the left ventricular wall. A possible etiology of the chordal rupture in this case was thought to be the excessive stretching caused by the disproportional visceromegaly of the heart.

  7. [Myopathy in acromegaly. Report of two cases].

    Science.gov (United States)

    Abe, M; Tabuchi, K; Fujii, K; Oda, K; Ishimoto, S

    1990-10-01

    Acromegaly is often associated with neuromuscular disorders. Most of them are caused by compression of nerves with hypertrophic bone and soft tissues or complications of diabetes mellitus. Myopathy has rarely been reported in the Japanese literature. We report two cases with myopathy out of 14 cases of acromegaly. Case 1 is a 62-year-old woman who developed muscle weakness and atrophy in the shoulder girdle, pelvic girdle and femoral regions after a 10-year history of acromegaly. She showed positive Gowers' sign and normal DTRs. Basal growth hormone (GH) level in plasma was 1076 ng/ml. Electromyograms (EMG) obtained from the deltoid and rectus femoris muscles revealed typical myopathic abnormalities; an excess of small-amplitude, short-duration, polyphasic motor unit potentials. Histological examinations of the rectus femoris muscle showed diffuse atrophy of both type I and type II fibers. She also had bilateral carpal tunnel syndrome and bilateral tarsal tunnel syndrome, which were confirmed by nerve conduction studies of median nerves and posterior tibial nerves. A cranial computed tomography (CT) scan demonstrated sellar mass with suprasellar extension. She underwent transsphenoidal adenomectomy and radiation therapy. GH level lowered to 29 ng/ml, however, myopathy remained unchanged for 3 years after the surgery. Case 2 is a 38-year-old woman who had undergone partial removal of a pituitary adenoma 9 years after the onset of acromegaly. Basal GH level in plasma before the surgery had been 1694 ng/ml and was still high after the surgery (100-505 ng/ml). The patient developed proximal muscle weakness and atrophy 4 years after the surgery.(ABSTRACT TRUNCATED AT 250 WORDS)

  8. [Carcinoma of the hypophysis with acromegaly].

    Science.gov (United States)

    Dusková, J; Chlumská, A; Vilikusová, E; Marek, J; Sprincl, L

    1984-08-01

    A 38-year old man deceased after being operated on recurring pituitary adenoma removed 13 years ago. Autopsy showed a massive spread of tumour on dura and in subarachnoid space. Growth hormone producing cells were found prevailing in the primary tumour as well as in secondaries by immunohistochemistry and immunofluorescence which concurred with acromegaly. Many cells produced prolactin. The tumour spreading discontinually in subdural and subarachnoid space was taken for a carcinoma.

  9. Glioblastoma multiforme after radiotherapy for acromegaly

    Energy Technology Data Exchange (ETDEWEB)

    Piatt, J.H. Jr.; Blue, J.M.; Schold, S.C. Jr.; Burger, P.C.

    1983-07-01

    A case of glioblastoma multiforme that occurred 14 years after radiotherapy for acromegaly is presented. The striking correspondence between the anatomy of the tumor and the geometry of the radiation ports is suggestive of a causal relationship. Previously reported cases of radiation-associated glioma are reviewed, and a brief appraisal of the evidence for induction of these lesions by radiation is presented. The differentiation of radiation-associated neoplasms from radionecrosis is also discussed.

  10. The evaluation of central corneal thickness and intraocular pressure in conjunction with tear IGF-1 levels in patients with acromegaly.

    Science.gov (United States)

    Kan, Emrah; Kan, Elif K; Okuyucu, Ali

    2017-08-30

    To compare the central corneal thickness (CCT), intraocular pressure (IOP), and tear insulin-like growth factor 1 (IGF-1) levels between patients with acromegaly and a control group and to evaluate the possible effect of tear IGF-1 and duration of the disease on CCT and IOP. We included 31 patients with acromegaly (study group) and 40 age- and sex-matched controls in the study. Patients with acromegaly were divided into 2 subgroups based on disease status (active/inactive). All participants underwent complete ophthalmologic evaluation including CCT and IOP values. Basal tear samples were collected from both groups and tear IGF-1 levels were measured. The CCT, IOP, and tear IGF-1 levels were compared between groups and subgroups and the association between tear IGF-I levels and ocular parameters (CCT, IOP) and disease duration were also evaluated. Central corneal thickness, IOP, and tear IGF-1 levels did not show a significant difference between study and control groups. We also did not find a significant difference in terms of CCT, IOP, or tear IGF-1 levels between subgroups of patients. Correlation analysis did not show an association between the duration of disease and tear IGF-1 levels with CCT or IOP. There was no significant difference in tear IGF-1 levels between patients with acromegaly and controls. Additionally, there was no correlation between disease duration and tear IGF-1 levels with CCT or IOP levels. This lack of association may suggest that tear IGF-1 levels might not have an effect on CCT or IOP findings in patients with acromegaly.

  11. Depression in acromegaly treated with escitalopram and cognitive therapy

    Directory of Open Access Journals (Sweden)

    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.

  12. A consensus on criteria for cure of acromegaly

    NARCIS (Netherlands)

    A. Giustina (Andrea); P. Chanson (Philippe); M.D. Bronstein; A. Klibanski; S.W.J. Lamberts (Steven); F.F. Casanueva; P. Trainer; E. Ghigo (Ezio); K.K.Y. Ho; S. Melmed (Shlomo)

    2010-01-01

    textabstractObjective: The Acromegaly Consensus Group met in April 2009 to revisit the guidelines on criteria for cure as defined in 2000. Participants: Participants included 74 neurosurgeons and endocrinologists with extensive experience of treating acromegaly. Evidence/Consensus Process: Relevant

  13. Combined treatment of somatostatin analogues with pegvisomant in acromegaly

    NARCIS (Netherlands)

    S.E. Franck (Sanne); A. Muhammad (Ammar); A-J. van der Lely (Aart-Jan); S.J.C.M.M. Neggers (Bas)

    2016-01-01

    textabstractTreatment 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

  14. Combined treatment of somatostatin analogues with pegvisomant in acromegaly

    NARCIS (Netherlands)

    S.E. Franck; A. Muhammad; A-J. van der Lely (Aart-Jan); S.J.C.M.M. Neggers (Bas)

    2016-01-01

    textabstractTreatment 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 ha

  15. A consensus on criteria for cure of acromegaly

    NARCIS (Netherlands)

    A. Giustina (Andrea); P. Chanson (Philippe); M.D. Bronstein; A. Klibanski; S.W.J. Lamberts (Steven); F.F. Casanueva; P. Trainer; E. Ghigo (Ezio); K.K.Y. Ho; S. Melmed (Shlomo)

    2010-01-01

    textabstractObjective: The Acromegaly Consensus Group met in April 2009 to revisit the guidelines on criteria for cure as defined in 2000. Participants: Participants included 74 neurosurgeons and endocrinologists with extensive experience of treating acromegaly. Evidence/Consensus Process: Relevant

  16. The Role of Radiotherapy in Acromegaly.

    Science.gov (United States)

    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.

  17. Current pharmacotherapy for acromegaly: a review.

    Science.gov (United States)

    Biermasz, Nienke R; Romijn, Johannes A; Pereira, Alberto M; Roelfsema, Ferdinand

    2005-11-01

    Acromegaly is associated with considerable morbidity and excess mortality; however, after effective treatment, both morbidity and mortality risks improve. Growth hormone excess in acromegaly can be controlled in many patients by pharmacotherapy alone, and with a combination of transsphenoidal surgery and pharmacotherapy in almost all patients. Since the clinical introduction of pegvisomant, a growth hormone-receptor antagonist, the role of radiotherapy is restricted. This review focuses on the treatment options for acromegaly (e.g., surgery, radiotherapy and pharmacotherapy with the depot preparations of the somatostatin analogues octreotide long-acting release formulation, lanreotide slow-release formulation and lanreotide Autogel, the growth hormone antagonist pegvisomant and the dopamine agonist cabergoline). Pharmacological characteristics of these drugs and the clinical and adverse effects are discussed individually and in relation to the other treatment modalities. The evidence for biochemical goals aimed at during medical treatment and the costs of pharmacotherapy are discussed. A new treatment algorithm is proposed, in which the choice between primary medical treatment and primary surgery is individualised, dependent on adenoma size and extension, patient factors (age, preference for therapy, contraindication for surgery), surgical experience of the centre and octreotide sensitivity of the adenoma. The high cost of lifelong medical treatment, especially of pegvisomant, must be weighed against the cost of a single surgical procedure.

  18. [Current diagnosis and treatment of acromegaly].

    Science.gov (United States)

    Melgar, Virgilio; Espinosa, Etual; Cuenca, Dalia; Valle, Vanessa; Mercado, Moisés

    2015-01-01

    Acromegaly is a rare condition characterized by the excessive secretion of growth hormone (GH), usually by a pituitary adenoma. The clinical manifestations of acromegaly include enlarged hands, feet and face, headaches, arthralgias, fatigue and hyperhydrosis. This condition is also associated with comorbidities such as hypertension and diabetes in a significant proportion of patients and frequently compromises life quality and life expectancy. The biochemical diagnosis of acromegaly rests on the demonstration of an autonomous secretion of GH by means of the measurement of glucose-suppressed GH levels and the serum concentration of insulin like growth factor type 1 (IGF-1). The localizing method of choice is magnetic resonance image of the selar area, which in 70 % of the cases reveals the presence of a macroadenoma. Even though the primary treatment is usually the transsphenoidal resection of the adenoma, the majority of patients require a multimodal intervention that includes radiotherapy, as well as pharmacological therapy with somatostatin analogs and dopamine agonists. The latter approach has resulted in a significant reduction in mortality and in an improvement in the quality of life.

  19. Combination of Klinefelter Syndrome and Acromegaly

    Science.gov (United States)

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

    2016-01-01

    Abstract 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 (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

  20. Elevated circulating somatostatin levels in acromegaly.

    Science.gov (United States)

    Arosio, M; Porretti, S; Epaminonda, P; Giavoli, C; Gebbia, C; Penati, C; Beck-Peccoz, P; Peracchi, M

    2003-06-01

    GH increases hypothalamic somatostatin (SS) synthesis and secretion but it is unknown if chronic GH excess, as found in acromegaly, may influence circulating SS levels, that are mainly of enteropancreatic source and affect several gastrointestinal functions, including motility. Circulating SS occurs in several post-translational forms including somatostatin-14 (SS-14), somatostatin-28 (SS-28) and other small peptides. The aim of the present study was to characterize the fasting and postprandial pattern of plasma circulating somatostatin in normal subjects and patients with acromegaly. Fasting total SS and SS-28 levels were measured in 32 subjects, 16 acromegalic patients with a new diagnosis (A) (8 F, 8 M, median age 48) and 16 matched healthy volunteers (C) (8 F, 8 M, median age 45). SS was also determined after a standard solid-liquid meal (550 kCal) in 24 of the subjects (12 C and 12 A). Fasting SS and SS-28 were significantly higher in acromegalic patients as compared to healthy subjects. In the former, a positive correlation was found between IGF-I and SS levels (r = 0.525 p acromegaly. Excess GH/IGF-I could be a causal factor in somatostatin hypersecretion. Conceivably this abnormality might play a role in some alterations of gastrointestinal function of acromegalic patients such as prolonged bowel transit.

  1. Serum GH and IGF-I are significant determinants of bone turnover but not bone mineral density in active acromegaly: a prospective study of more than 70 consecutive patients.

    Science.gov (United States)

    Ueland, T; Fougner, S L; Godang, K; Schreiner, T; Bollerslev, J

    2006-11-01

    Acromegaly is characterized by a persistent hypersecretion of GH and provides information on long-term effects of GH on bone metabolism. The aim of this study was to examine the effect of gonadal status and disease activity on bone metabolism in active acromegaly. Seventy-three consecutive patients with active acromegaly: 40 women and 33 men (50 +/- 13 (mean +/- s.d.) and 49 +/- 10 years respectively) were evaluated and compared with age-, sex-, and body mass index (BMI)-matched controls by X-ray absorptiometry and biochemical analysis (markers of disease activity and bone turnover). We found that bone turnover, as evaluated by biochemical bone markers, is coupled and markedly increased in relation to disease activity in active acromegaly. Acromegalic women, but not men, were characterized by an increased bone area and slightly decreased bone mineral content resulting in significantly decreased bone mineral density (BMD) in the ultradistal radius, proximal radius, and total body. No differences in bone turnover or BMD were found between eu-and hypogonadal subjects. Multivariate analysis identified age, BMI, and gender as independent predictors of total BMD in acromegaly. Our study demonstrates a decreased total body BMD in women, not men, with active acromegaly, regardless of gonadal status or disease activity. Bone turnover is markedly increased in relation to disease activity, possibly counteracting the anabolic effects of excess GH/IGF-I in these subjects. We suggest more focus on biomechanical analyses when investigating endocrine disorders affecting bone size and distribution between compartments.

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

    Science.gov (United States)

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

    2013-01-01

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

  3. 5 Long-term acromegaly and associated cardiovascular complications: a case-based review.

    Science.gov (United States)

    Colao, Annamaria

    2009-12-01

    Because growth hormone and IGF-1 both have regulatory roles in the cardiovascular system, patients with acromegaly often present with abnormalities of heart structure and function and the vascular system, which if left unmanaged can reduce life expectancy. Early symptoms of acromegalic cardiomyopathy (hyperkinetic syndrome) can be characterized by cardiac hypertrophy, increased heart rate, and increased systolic output. When left untreated, more pronounced hypertrophy, signs of diastolic dysfunction and insufficient systolic function on exertion arise, and can lead to systolic dysfunction at rest, and eventually heart failure with signs of dilative cardiomyopathy. Increasingly, evidence suggests that early diagnosis and treatment of acromegaly (before the age of 40 years) can help prevent the progression of cardiovascular disease, improve quality of life, and reduce the risk of premature mortality. This review focuses on management strategies for newly diagnosed patients with acromegaly and evidence of cardiovascular disease. The roles of surgery and medical treatment are discussed in the context of using optimal treatment strategies to help reverse cardiac hypertrophy and normalize other cardiac risk factors.

  4. Spontaneous acromegaly: a retrospective case control study in German shepherd dogs.

    Science.gov (United States)

    Fracassi, F; Zagnoli, L; Rosenberg, D; Furlanello, T; Caldin, M

    2014-10-01

    Acromegaly results from the overproduction of growth hormone in adulthood and is characterised by overgrowth of soft tissue and/or bone as well as insulin resistance. There are few data indicating the risk factors associated with this disease in dogs or its clinicopathological features and sequelae. The objective of this retrospective study was to catalogue and assess these aspects of the disease in German shepherd dogs (GSDs) which were found to be over-represented among acromegalic dogs attending two veterinary referral clinics over a period of 7 years. Each acromegalic dog (AD) was compared with two breed/age/sex matched controls. Clinical signs of acromegaly included panting, polyuria/polydipsia, widened interdental spaces, weakness, inspiratory stridor, macroglossia, weight gain, redundant skin folds, thick coat, exophthalmos and mammary masses. Serum alkaline phosphatase, creatine-kinase, glucose, triglyceride, phosphate ion, and 'calcium per phosphate product' concentrations were significantly higher in acromegalic animals while haemoglobin concentration, blood urea nitrogen, sodium and chloride ion concentrations, and urinary specific gravity, osmolality and fractional excretion of phosphate were significantly lower. Although, in the majority of cases clinicopathological abnormalities resolved following ovariohysterectomy, in one dog, acromegalic signs abated and insulin-like growth factor-1 concentrations normalised only following the surgical excision of mammary tumours carried out 2 months after ovariohysterectomy. The findings of this study indicate that GSDs are predisposed to the development of acromegaly with a suspected inherited susceptibility.

  5. Increased Population Risk of AIP‐Related Acromegaly and Gigantism in Ireland

    Science.gov (United States)

    Radian, Serban; Diekmann, Yoan; Gabrovska, Plamena; Holland, Brendan; Bradley, Lisa; Wallace, Helen; Stals, Karen; Bussell, Anna‐Marie; McGurren, Karen; Cuesta, Martin; Ryan, Anthony W.; Herincs, Maria; Hernández‐Ramírez, Laura C.; Holland, Aidan; Samuels, Jade; Aflorei, Elena Daniela; Barry, Sayka; Dénes, Judit; Pernicova, Ida; Stiles, Craig E.; Trivellin, Giampaolo; McCloskey, Ronan; Ajzensztejn, Michal; Abid, Noina; Akker, Scott A.; Mercado, Moises; Cohen, Mark; Thakker, Rajesh V.; Baldeweg, Stephanie; Barkan, Ariel; Musat, Madalina; Levy, Miles; Orme, Stephen M.; Unterländer, Martina; Burger, Joachim; Kumar, Ajith V.; Ellard, Sian; McPartlin, Joseph; McManus, Ross; Linden, Gerard J.; Atkinson, Brew; Balding, David J.; Agha, Amar; Thompson, Chris J.; Hunter, Steven J.; Thomas, Mark G.; Morrison, Patrick J.

    2016-01-01

    ABSTRACT The aryl hydrocarbon receptor interacting protein (AIP) founder mutation R304* (or p.R304*; NM_003977.3:c.910C>T, p.Arg304Ter) identified in Northern Ireland (NI) predisposes to acromegaly/gigantism; its population health impact remains unexplored. We measured R304* carrier frequency in 936 Mid Ulster, 1,000 Greater Belfast (both in NI) and 2,094 Republic of Ireland (ROI) volunteers and in 116 NI or ROI acromegaly/gigantism patients. Carrier frequencies were 0.0064 in Mid Ulster (95%CI = 0.0027–0.013; P = 0.0005 vs. ROI), 0.001 in Greater Belfast (0.00011–0.0047) and zero in ROI (0–0.0014). R304* prevalence was elevated in acromegaly/gigantism patients in NI (11/87, 12.6%, P < 0.05), but not in ROI (2/29, 6.8%) versus non‐Irish patients (0–2.41%). Haploblock conservation supported a common ancestor for all the 18 identified Irish pedigrees (81 carriers, 30 affected). Time to most recent common ancestor (tMRCA) was 2550 (1,275–5,000) years. tMRCA‐based simulations predicted 432 (90–5,175) current carriers, including 86 affected (18–1,035) for 20% penetrance. In conclusion, R304* is frequent in Mid Ulster, resulting in numerous acromegaly/gigantism cases. tMRCA is consistent with historical/folklore accounts of Irish giants. Forward simulations predict many undetected carriers; geographically targeted population screening improves asymptomatic carrier identification, complementing clinical testing of patients/relatives. We generated disease awareness locally, necessary for early diagnosis and improved outcomes of AIP‐related disease. PMID:27650164

  6. PREVALENCE OF SLEEP DISORDERED BREATHING IN PATIENTS WITH NEWLY DIAGNOSED ACROMEGALY

    Directory of Open Access Journals (Sweden)

    U. A. Tsoy

    2014-01-01

    Full Text Available Background: Obstructive sleep disordered breathing or obstructive sleep apnea (OSA is the most common respiratory impairment in acromegaly. OSA is bound up with heightened cardiovascular mortality. Aim: Тo study frequency, features, and structure of sleep disordered breathing in patients with newly diagnosed acromegaly and to elucidate the factors influencing their development. Materials and methods: 38 patients (10 men, 28 women, median age 53 (28-76 years, median body mass index (BMI 29 (19.9-44.3 kg/m² with newly diagnosed acromegaly were recruited into the study. All subjects underwent full polysomnography (Embla N7000, Natus, USA and Remlogica software (USA. Results: Sleep disordered breathing was found in 28 (73.7% patients. OSA was revealed in all cases, in 11 (39.3% subjects it was mixed. In 10 (35.7% patients OSA was mild, in 8 (28.6% moderate, and in 10 (35.7% severe. BMI (р<0.01, disease duration (р=0.003, and insulin-like growth factor-1 (IGF-1 level (р=0.04 were different in patients without OSA and patients with moderate-to-severe OSA. No difference was found in sex (р=0.4, age (р=0.064, and growth hormone level (р=0.6. Frequency of arterial hypertension, diabetes mellitus, and other glucose metabolism impairments was the same in subjects without OSA and with severe-to-moderate OSA. Conclusion: All patients with newly diagnosed acromegaly should undergo polysomnography. BMI, disease duration, and IGF-1 level are significant risk factors for OSA development. Correlation OSA with arterial hypertension and glucose metabolism impairments needs to be further investigated.

  7. Polycythemia as rare secondary direct manifestation of acromegaly: management and single-centre epidemiological data.

    Science.gov (United States)

    Zoppoli, Gabriele; Bianchi, Federico; Bruzzone, Andrea; Calvia, Alessandro; Oneto, Caterina; Passalia, Caterina; Balleari, Enrico; Bedognetti, Davide; Ponomareva, Elena; Nazzari, Elena; Castelletti, Lara; Castellan, Lucio; Minuto, Francesco; Ghio, Riccardo; Ferone, Diego

    2012-06-01

    Polycythemia associated with acromegaly is usually caused by the systemic manifestations of the disease, such as sleep-apnea or concomitant erythropoietin-secreting kidney tumors. The recognition of underlying pathologies requires a thorough diagnostic process. We report a unique case of acromegaly with polycythemia, not caused by commonly described manifestations of the disease, and receding with octreotide therapy. The medical history of 141 acromegalic patients followed by the Endocrinology Unit of the San Martino University Hospital in Genoa has been also reviewed, together with the literature evidence for similar cases. The diagnostic workflow and 2-years follow-up of a 43-years old acromegalic, polycythemic man with a history of past smoking, moderate hypertension, and mental retardation are described. The hematological parameters of our cohort was retrospectively compared with those of a healthy, age/gender-related control group as well. Therapy with octreotide LAR, 20 mg i.m. q28d was begun soon after diagnosis of acromegaly in the polycythemic patient. Haematocrit level, hormonal setting, as well as pituitary tumor size and visual perimetry during treatment were recorded. Octreotide LAR treatment normalized hormonal alterations, as well as hematological parameters. Polycythemia has not recurred after 2 years of therapy. The median hemoglobin and hematocrit levels of the retrospectively analyzed cohort of acromegalic were significantly lower than normal ranges of a healthy, age/sex- related control population. In conclusions, polycythemia can be a direct, albeit rare, secondary manifestation of acromegaly, that must be considered during the diagnostic work-up of acromegalic patients presenting with such disorder.

  8. [Salivary cortisol among subjects with and without Cushing syndrome].

    Science.gov (United States)

    Lépez, Macarena; Caamaño, Egardo; Romero, Carmen; Fiedler, Jenny; Araya, Verónica

    2010-02-01

    Salivary cortisol measurement is recommended as a screening mea-sure when a Cushing Syndrome is suspected. The proposed cut-off point for a probably diagnosis is 0.16 ug/dL. To determine salivary cortisol concentrations during the day inpatients with and without Cushing syndrome and with depression. Salivary cortisol was measured by competitive enzyme immuno assay (EIA), in samples obtained at 8:00,15:00 and 23:00 h in 78 patients without Cushing syndrome, aged 40 +/- 15 years (28 males), 30 patients with depression aged 40 +/- 12 years (nine males) and four females with Cushing syndrome aged 42 +/- 17 years. Salivary cortisol was higher among patients with Cushing syndrome than the rest of patients. A salivary cortisol over the cut-off value of O.16 ug/dL was found in 42% of subjects without Cushing syndrome and in 33% of patients with depression. Median values among patients without Cushing syndrome, depression and with Cushing syndrome were 0.21 (range Cushing syndrome but there was a great overlap with values obtained in subjects without the syndrome.

  9. ASYMMETRIC DIMETHYLARGININE LEVELS AND ATHEROSCLEROSIS MARKERS IN CUSHING SYNDROME.

    Science.gov (United States)

    Ozsurekci, Cemile Gulbas; Akturk, Mujde; Ozkan, Cigdem; Gulbahar, Ozlem; Altinova, Alev Eroglu; Yalcin, Muhittin; Arslan, Emre; Toruner, Fusun

    2016-09-01

    As a consequence of hypercortisolism, Cushing syndrome (CS) is frequently observed with other diseases that are associated with atherosclerosis, including diabetes mellitus, dyslipidemia, hypertension, and obesity. Cardiovascular disease (CVD) is the primary cause of mortality and morbidity in CS. We investigate CVD risk markers such as asymmetric dimethylarginine (ADMA), lipoprotein-associated phospholipase A2 (Lp-PLA2), highsensitive C-reactive protein (hsCRP), homocysteine, lipid levels, ankle-brachial index (ABI), and carotid intimamedia thickness (CIMT) in CS. Our study included 27 patients with CS and 27 age-, sex-, body mass index (BMI)-, and comorbid disease-matched control subjects. Plasma ADMA levels were significantly lower in the CS group than the control group (P = .013). Total cholesterol, low-density lipoprotein, triglycerides, high-density lipoprotein, and apolipoprotein A1 and apolipoprotein B levels were higher in patients with CS than the control group (P.05). We found that ADMA levels were lower in CS, the finding that should be further investigated. Levels of hsCRP, Lp-PLA2, and homocysteine levels and CIMT and ABI measurements were similar between the CS group and comorbidity-matched control group. None of these markers was prominent to show an increased risk of CVD in CS, independent of the comorbidities of CS. ABI = ankle-brachial index Apo = apolipoprotein ADMA = asymmetric dimethylarginine BMI = body mass index CVD = cardiovascular disease CIMT = carotid intima-media thickness CS = Cushing syndrome DM = diabetes mellitus DDAH = dimethylarginine dimethylaminohydrolase ELISA = enzyme-linked immunosorbent assay HDL = high-density lipoprotein hsCRP = high-sensitive C-reactive protein HOMA-IR = homeostatic model assessment of insulin resistance HT = hypertension LDL = low-density lipoprotein Lp-PLA2 = lipoprotein-associated phospholipase A2 Lp-a = lipoprotein a NO = nitric oxide.

  10. Increased Short-Term Beat-To-Beat Variability of QT Interval in Patients with Acromegaly.

    Science.gov (United States)

    Orosz, Andrea; Csajbók, Éva; Czékus, Csilla; Gavallér, Henriette; Magony, Sándor; Valkusz, Zsuzsanna; Várkonyi, Tamás T; Nemes, Attila; Baczkó, István; Forster, Tamás; Wittmann, Tibor; Papp, Julius Gy; Varró, András; Lengyel, Csaba

    2015-01-01

    Cardiovascular diseases, including ventricular arrhythmias are responsible for increased mortality in patients with acromegaly. Acromegaly may cause repolarization abnormalities such as QT prolongation and impairment of repolarization reserve enhancing liability to arrhythmia. The aim of this study was to determine the short-term beat-to-beat QT variability in patients with acromegaly. Thirty acromegalic patients (23 women and 7 men, mean age±SD: 55.7±10.4 years) were compared with age- and sex-matched volunteers (mean age 51.3±7.6 years). Cardiac repolarization parameters including frequency corrected QT interval, PQ and QRS intervals, duration of terminal part of T waves (Tpeak-Tend) and short-term variability of QT interval were evaluated. All acromegalic patients and controls underwent transthoracic echocardiographic examination. Autonomic function was assessed by means of five standard cardiovascular reflex tests. Comparison of the two groups revealed no significant differences in the conventional ECG parameters of repolarization (QT: 401.1±30.6 ms vs 389.3±16.5 ms, corrected QT interval: 430.1±18.6 ms vs 425.6±17.3 ms, QT dispersion: 38.2±13.2 ms vs 36.6±10.2 ms; acromegaly vs control, respectively). However, short-term beat-to-beat QT variability was significantly increased in acromegalic patients (4.23±1.03 ms vs 3.02±0.80, Pacromegaly in spite of unchanged conventional parameters of ventricular repolarization. This enhanced temporal QT variability may be an early indicator of increased liability to arrhythmia.

  11. Increased Short-Term Beat-To-Beat Variability of QT Interval in Patients with Acromegaly

    Science.gov (United States)

    Orosz, Andrea; Csajbók, Éva; Czékus, Csilla; Gavallér, Henriette; Magony, Sándor; Valkusz, Zsuzsanna; Várkonyi, Tamás T.; Nemes, Attila; Baczkó, István; Forster, Tamás; Wittmann, Tibor; Papp, Julius Gy.; Varró, András; Lengyel, Csaba

    2015-01-01

    Cardiovascular diseases, including ventricular arrhythmias are responsible for increased mortality in patients with acromegaly. Acromegaly may cause repolarization abnormalities such as QT prolongation and impairment of repolarization reserve enhancing liability to arrhythmia. The aim of this study was to determine the short-term beat-to-beat QT variability in patients with acromegaly. Thirty acromegalic patients (23 women and 7 men, mean age±SD: 55.7±10.4 years) were compared with age- and sex-matched volunteers (mean age 51.3±7.6 years). Cardiac repolarization parameters including frequency corrected QT interval, PQ and QRS intervals, duration of terminal part of T waves (Tpeak-Tend) and short-term variability of QT interval were evaluated. All acromegalic patients and controls underwent transthoracic echocardiographic examination. Autonomic function was assessed by means of five standard cardiovascular reflex tests. Comparison of the two groups revealed no significant differences in the conventional ECG parameters of repolarization (QT: 401.1±30.6 ms vs 389.3±16.5 ms, corrected QT interval: 430.1±18.6 ms vs 425.6±17.3 ms, QT dispersion: 38.2±13.2 ms vs 36.6±10.2 ms; acromegaly vs control, respectively). However, short-term beat-to-beat QT variability was significantly increased in acromegalic patients (4.23±1.03 ms vs 3.02±0.80, Pacromegaly in spite of unchanged conventional parameters of ventricular repolarization. This enhanced temporal QT variability may be an early indicator of increased liability to arrhythmia. PMID:25915951

  12. The role of combination medical therapy in the treatment of acromegaly.

    Science.gov (United States)

    Lim, Dawn Shao Ting; Fleseriu, Maria

    2017-02-01

    Uncontrolled acromegaly results in approximately 2-fold excess mortality. Pituitary surgery is first-line therapy, and medical treatment is indicated for persistent disease. While cabergoline and pegvisomant are used in select patients, somatostatin receptor ligands (SRLs) remain the cornerstone of medical treatment. Management of patients poorly responsive to SRLs is therefore, challenging. The purpose of this review is to highlight the options for combination medical therapy in the treatment of acromegaly, with an emphasis on efficacy and safety. All original articles/abstracts detailing combination medical therapy in acromegaly were identified from a PubMed search. Studies reviewed included retrospective and open-label prospective studies. While the combination of SRL and cabergoline was generally well tolerated, a lower baseline insulin-like growth factor-1 (IGF-1) level was the best predictor of efficacy; this combination may be most effective in patients with mildly elevated IGF-1. SRL-pegvisomant combination normalized IGF-1 in the majority of patients; continued efficacy despite individual drug dosing reduction was also reported. The risk of significant liver enzyme elevation was, however, higher than that reported with SRL monotherapy; close monitoring is recommended. Data on pegvisomant-cabergoline combination is limited, but this may be an option in the setting of SRL intolerance. Reports on temozolomide used in combination with other medical therapies in patients with aggressive GH-secreting tumors are also summarized. While more prospective, randomized controlled trials on long-term efficacy and safety are needed, combination medical therapy remains a treatment strategy that should be considered for acromegaly patients poorly responsive to SRLs.

  13. The effects of somatostatin analogue therapy on pituitary tumor volume in patients with acromegaly.

    Science.gov (United States)

    Colao, Annamaria; Auriemma, Renata S; Pivonello, Rosario

    2016-04-01

    In nearly all cases, acromegaly is caused by excess GH from a pituitary adenoma, resulting in elevated circulating levels of GH and, subsequently, IGF-1. Treatment goals are to eliminate morbidity and restore the increased mortality to normal rates. Therapeutic strategies aim to minimize tumor mass and normalize GH and IGF-1 levels. Somatostatin analogues are the medical treatment of choice in acromegaly, as first-line or post-surgical therapy, and have proven efficacy in pituitary tumor volume reduction (TVR). Here we review the effects of somatostatin analogue therapy on pituitary tumor volume in patients with acromegaly. TVR with somatostatin analogues may be mediated by direct anti-proliferative effects via activation of somatostatin receptors, or by indirect effects, such as angiogenesis inhibition, and is more pronounced when they are administered as first-line therapy. Various studies of first-line treatment with octreotide LAR have shown significant TVR in ≥73% of patients. First-line treatment with lanreotide Autogel has shown evidence of TVR, although more studies are needed. In a recent randomized, double-blind, 12-month trial in 358 medical-treatment-naïve acromegaly patients, significant TVR was achieved by 81% of patients administered pasireotide LAR and 77% administered octreotide LAR. Pre-operative somatostatin analogue therapy may also induce TVR and improve post-operative disease control compared with surgery alone. TVR is progressive with prolonged treatment, and decreased IGF-1 levels may be its best predictor, followed by age and degree of GH decrease. However, TVR does not always correlate with degree of biochemical control. Somatostatin analogues (first- or second-line treatment) are the mainstay of medical therapy and, as first-line medical therapy, are associated with significant pituitary TVR in most patients.

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

    Directory of Open Access Journals (Sweden)

    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.

  15. A risk-benefit assessment of octreotide in the treatment of acromegaly.

    Science.gov (United States)

    van der Lely, A J; de Herder, W W; Lamberts, S W

    1997-11-01

    Acromegaly was the first pituitary disease to be recognised as a clinical entity, although initially it was not clear whether the eosinophilic adenomas causing pituitary enlargement were causative or just a manifestation of the syndrome itself. Following the documented clinical improvement of patients with acromegaly after partial hypophysectomy, it was proven that the pituitary adenomas were aetiological. The treatment of acromegaly has changed during the last decades; the introduction of the somatostatin (SMS) analogue octreotide has had major implications. Octreotide was the first SMS analogue to become available for clinical use. It is generally well tolerated, but is associated with the development of gallstones in 15 to 20% of patients. Other adverse effects include transient injection-site pain, abdominal, diarrhoea, gastritis (long term therapy) and loss of scalp hair. No long haematological or biochemical adverse effects have been reported. Desensitisation to the beneficial effects of octreotide therapy is highly unusual. A long-acting formulation of octreotide is being studied, and should be available by the end of 1997.

  16. [Suppression by dopamine of GH release induced by GRF in a case of acromegaly].

    Science.gov (United States)

    Matsubara, M; Odagaki, E; Morioka, T

    1987-03-20

    Inhibition of plasma GH by dopaminergic agonists is one of the characteristics of the GH secretion in acromegaly. GRF is known to stimulate GH secretion in most patients with acromegaly. In order to elucidate the relationship between GRF and dopamine in regulating the secretion of GH in this disease, we examined plasma GH responses to dopamine (DA) infusion (4 micrograms/kg/min), GRF injection (100 micrograms i.v.), sulpiride (SP) injection (200 mg i.v.), a DA blocker, DA plus GRF and SP plus GRF in a 51-year-old male patient with acromegaly. Plasma GH was reduced to 14% of the initial level by iv infusion of DA, and was elevated to 158% by iv injection of GRF. No considerable change was observed in plasma GH by iv infusion of SP (114% of the initial level). GH release induced by GRF was remarkably reduced by simultaneous administration of DA (28% of the initial level), whereas SP administration did not affect GRF-induced GH release (154%). The marked reduction of GH release after DA plus GRF seems to suggest that the effect of DA on the GH regulation is stronger than that of GRF in this acromegalic patient. It is suggested also that endogenous DA may not play an inhibitory role in GH secretion in this case since DA blockade by SP did not raise basal GH levels and the GH response to GRF.

  17. Treatment of acromegaly increases BMD but reduces trabecular bone score: a longitudinal study.

    Science.gov (United States)

    Godang, Kristin; Olarescu, Nicoleta Cristina; Bollerslev, Jens; Heck, Ansgar

    2016-08-01

    Bone turnover is increased in acromegaly. Despite normalization of bone turnover after treatment, the risk for vertebral fractures remains increased. Gonadal status, but not BMD, is correlated with vertebral fractures. Trabecular bone score (TBS) is related to bone microarchitecture. The aim of this study is to assess the longitudinal change in TBS and BMD following treatment for acromegaly. DESIGN, SETTING, PATIENTS, INTERVENTIONS, AND MAIN OUTCOME MEASURES: This longitudinal study included 48 patients with acromegaly between 2005 and 2015. BMD, TBS, and markers for bone turnover (P1NP and CTX-1) were measured at baseline and following treatment. Following treatment, the mean TBS decreased by 3.0 (±7.0) %, whereas the BMD at the lumbar spine (LS) increased by 3.2 (±4.9) % (both Pacromegaly affects TBS and BMD at LS in different manners. The reduction of bone turnover markers predicts the increase in BMD but not the decrease in TBS. The DXA changes were more pronounced in men. Alterations in trabecular bone architecture may explain the persistent fracture risk despite the increase in BMD after disease control. © 2016 European Society of Endocrinology.

  18. Ectopic acromegaly due to a growth hormone-secreting neuroendocrine-differentiated tumor developed from ovarian mature cystic teratoma.

    Science.gov (United States)

    Ozkaya, Mesut; Sayiner, Zeynel Abidin; Kiran, Gurkan; Gul, Kamile; Erkutlu, Ibrahim; Elboga, Umut

    2015-06-01

    Acromegaly is a clinical syndrome caused by the overproduction of growth hormone (GH) and also known as a rare disease. Clinical, biochemical, and radiological features are often indistinguishable between GH-producing hypophysis adenomas and ectopic GH-releasing hormone (GHRH)-producing tumors. A 40-year-old woman presented to us with her growing feet, hands especially fingers, and enlarging nose. Biochemical diagnosis of acromegaly was made by measuring insulin-like growth factor-1 (IGF-1) level and glucose-suppressed GH estimation. Her spot IGF-1 level was 1300 ng/ml (90-226 ng/ml). The basal GH was 30 ng/l, and 60- and 120-min GH levels after 75-g oral glucose load were 29 and 40 ng/l, respectively. Magnetic resonance imaging (MRI) of pituitary was normal. There was no pituitary adenoma or pituitary hyperplasia. Extrapituitary ectopic hypersecretion of GH or GHRH-secreting tumor search was done by high-resolution computed tomography (CT) of chest and whole abdomen. Abdomen CT revealed 9.5 × 8 cm pelvic mass, which included calcific regions and solid component. The specimen's immunohistochemical staining with GH was positive but interestingly GHRH was negative. According to immunohistochemical staining, the patient's diagnosis was ectopic acromegaly due to a GH-secreting neuroendocrine-differentiated tumor developed from an ovarian mature cystic teratoma. Herein, we present excellent illustration of an unusual and confusing clinical scenario of ectopic acromegaly.

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

    Science.gov (United States)

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

    2015-01-01

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

  20. Investigation of the Vitamin D Receptor Polymorphisms in Acromegaly Patients

    Science.gov (United States)

    Ilhan, Muzaffer; Toptas-Hekimoglu, Bahar; Yaylim, Ilhan; Turgut, Seda; Turan, Saime; Karaman, Ozcan; Tasan, Ertugrul

    2015-01-01

    Objective. The genetic structural alterations in the majority of somatotroph adenomas are not clarified and the search for novel candidate genes is still a challenge. We aimed to investigate possible associations between vitamin D receptor (VDR) polymorphisms and acromegaly. Design, Patients, and Methods. 52 acromegaly patients (mean age 45.7 ± 1.9 years) and 83 controls (mean age 43.1 ± 2.6 years) were recruited to the study. VDR polymorphism was determined by polymerase chain reaction-based restriction fragment length polymorphism methods. Results. The distribution of VDR genotypes showed a significant difference in the frequencies of VDR FokI genotypes between patients and controls (P = 0.034). VDR FokI ff genotype was significantly decreased in acromegaly patients (P = 0.035) and carriers of FokI Ff genotype had a 1.5-fold increased risk for acromegaly (OR: 1.5, 95% CI: 1.07–2.1; P = 0.020). IGF1 levels after treatment were significantly higher in patients carrying the Ff genotype compared to carrying ff genotype (P = 0.0049). 25(OH)D3 levels were significantly lower in acromegaly patients (P < 0.001). Conclusions. Our study suggests that VDR FokI genotypes might affect the development of acromegaly and VDR polymorphisms may play a role in the course of acromegaly as a consequence of altering hormonal status. PMID:25839036

  1. Investigation of the Vitamin D Receptor Polymorphisms in Acromegaly Patients

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    Muzaffer Ilhan

    2015-01-01

    Full Text Available Objective. The genetic structural alterations in the majority of somatotroph adenomas are not clarified and the search for novel candidate genes is still a challenge. We aimed to investigate possible associations between vitamin D receptor (VDR polymorphisms and acromegaly. Design, Patients, and Methods. 52 acromegaly patients (mean age 45.7±1.9 years and 83 controls (mean age 43.1±2.6 years were recruited to the study. VDR polymorphism was determined by polymerase chain reaction-based restriction fragment length polymorphism methods. Results. The distribution of VDR genotypes showed a significant difference in the frequencies of VDR FokI genotypes between patients and controls (P=0.034. VDR FokI ff genotype was significantly decreased in acromegaly patients (P=0.035 and carriers of FokI Ff genotype had a 1.5-fold increased risk for acromegaly (OR: 1.5, 95% CI: 1.07–2.1; P=0.020. IGF1 levels after treatment were significantly higher in patients carrying the Ff genotype compared to carrying ff genotype (P=0.0049. 25(OHD3 levels were significantly lower in acromegaly patients (P<0.001. Conclusions. Our study suggests that VDR FokI genotypes might affect the development of acromegaly and VDR polymorphisms may play a role in the course of acromegaly as a consequence of altering hormonal status.

  2. Síndrome de Cushing e Gravidez

    OpenAIRE

    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.

  3. Subclinical Cushing's syndrome: current concepts and trends.

    Science.gov (United States)

    Zografos, George N; Perysinakis, Iraklis; Vassilatou, Evangeline

    2014-01-01

    Clinically inapparent adrenal masses which are incidentally detected have become a common problem in everyday practice. Approximately 5-20% of adrenal incidentalomas present subclinical cortisol hypersecretion which is characterized by subtle alterations of the hypothalamic-pituitary-adrenal axis due to adrenal autonomy. This disorder has been described as subclinical Cushing's syndrome, since there is no typical clinical phenotype. The diagnosis of subclinical Cushing's syndrome is based on biochemical evaluation; however, there is still no consensus for the biochemical diagnostic criteria. An abnormal 1mg dexamethasone suppression test (DST) as initial screening test in combination with at least one other abnormal test of the hypothalamic-pituitary-adrenal axis has been advocated by most experts for the diagnosis of subclinical Cushing's syndrome. DST is the main method of establishing the diagnosis, while there is inhomogeneity of the information that other tests provide. Arterial hypertension, diabetes mellitus type 2 or impaired glucose tolerance, central obesity, osteoporosis/vertebral fractures and dyslipidemia are considered as detrimental effects of chronic subtle cortisol excess, although there is no proven causal relationship between subclinical cortisol hypersecretion and these morbidities. Therapeutic strategies include careful observation along with medical treatment of morbidities potentially related to subtle cortisol hypersecretion versus laparoscopic adrenalectomy. The optimal management of patients with subclinical Cushing's syndrome is not yet defined. The conservative approach is appropriate for the majority of these patients; however, the duration of follow-up and the frequency of periodical evaluation still remain open issues. Surgical resection may be beneficial for patients with hypertension, diabetes mellitus type 2 or abnormal glucose tolerance and obesity.

  4. Cross-sectional prevalence of pancreatic cystic lesions in patients with acromegaly, a single-center experience.

    Science.gov (United States)

    Odake, Yukiko; Fukuoka, Hidenori; Yamamoto, Masaaki; Arisaka, Yoshifumi; Konishi, Junya; Yoshida, Kenichi; Matsumoto, Ryusaku; Bando, Hironori; Suda, Kentaro; Nishizawa, Hitoshi; Iguchi, Genzo; Yamada, Shozo; Ogawa, Wataru; Takahashi, Yutaka

    2017-05-24

    Acromegaly is a disease associated with an increased risk for several kinds of neoplasms including colon and thyroid cancer. Although the association between acromegaly and pancreatic neoplasms has not been elucidated, it has recently been reported that GNAS gene mutations were found in 58% of intraductal papillary mucinous neoplasms (IPMNs), which are representative pancreatic cystic lesions, suggesting a link between IPMNs and acromegaly. To assess the prevalence of pancreatic cystic lesions in patients with acromegaly, we performed a retrospective cross-sectional single institute study. Thirty consecutive acromegalic patients (20 females and 10 males; mean age, 60.9 ± 11.9 years) who underwent abdominal contrast-enhanced computed tomography or magnetic resonance imaging between 2007 and 2015 at Kobe University Hospital were recruited. We also analyzed the relationship between presence of pancreatic cystic lesions and somatic GNAS mutations in pituitary tumors. Seventeen of 30 (56.7%) patients studied had pancreatic cystic lesions. Nine of 17 patients (52.9%) were diagnosed with IPMNs based on imaging findings. These results suggest that the prevalence of IPMNs may be higher in acromegalic patients in acromegalic patients than historically observed in control patients (up to 13.5%). In patients with pancreatic cystic lesions, the mean patient age was higher and the duration of disease was longer than in those without pancreatic cystic lesions (67.0 ± 2.3 vs. 53.0 ± 2.7 years, p lesions. These data demonstrate that old or long-suffering patients with acromegaly have a higher prevalence of pancreatic cystic lesions. Moreover, the prevalence of pancreatic cystic lesions may be increased in acromegalic patients.

  5. Low Prevalence of Periodontitis in Acromegaly: Growth Hormone May Exert a Protective Effect

    Directory of Open Access Journals (Sweden)

    Hülya Serinsöz

    2015-06-01

    Full Text Available Purpose: To evaluate bone mineral density (BMD measurements and the presence of periodontitis in patients with acromegaly, as well as to inquire the impact of interfering factors. Material and Method: Forty-seven acromegalic patients with any accompanying condition known to affect calcium-bone metabolism and 60 age-matched healthy controls were included. Age, gender, duration and activity of acromegaly, past-present therapy options, pituitary hormone profiles, replacement therapies, and the results of periodontal analysis were recorded. Results: Eighteen patients were male (38.3%, 29 were female (61.7%. The mean age of the patients was 46.6±11.5 years, twenty-five (53.1% had active, 22 (46.8% had inactive acromegaly. The latter were older and had longer disease duration (p=0.04, p=0.003, respectively. Serum calcium and phosphorus levels, 24-hour urinary calcium excretion and BMD at the lumbar spine and femur neck insignificantly associated with disease activity (p>0.05. Osteoporosis was detected in 6 patients (12.76%. Periodontitis and advanced periodontitis were more common in control group (66.7% vs. 44.7%, (43.3% vs. 12.8% (p=0.022, p=0.0001, respectively. There was no difference in chronic periodontitis and severity between active and inactive groups (48% vs. 40.9%; p=0.279. No difference was noted in other study parameters, as well. Repeated measures analysis of variance demonstrated statistically insignificant distribution between GH change in time and periodontitis subgroups. Discussion: We demonstrated that acromegaly exerted no clear negative impact on vertebral BMD in the absence of overt hypogonadism. Regardless of disease activity, acromegaly cases exhibited lower rates of periodontitis with less severity which remained unchanged in the presence of accompanying metabolic disorders known to have negative impact on periodontal tissue. Chronic exposure to excess GH may have a protective role against periodontitis. Turk Jem 2015; 19: 42-48

  6. Association of Acromegaly and Multiple Myeloma: A Case Report

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    Murat Atmaca

    2013-09-01

    Full Text Available Malignancy is an important cause of mortality in acromegaly. Hematological malignancies are very rare in acromegaly. Here, we report an 80-year-old patient with acromegaly and multiple myeloma. Patient died within a month of diagnosis. Previous studies have shown that growth hormone and somatomedin-C activate B lymphocyte and somatomedin-C receptors are found in multiple myeloma cells. Possible effects of growth hormone and somatomedin-C on multiple myeloma progression are discussed in the light of the relevant literature. Turk Jem 2013; 17: 75-7

  7. Outcome in acromegaly: A retrospective analysis

    Directory of Open Access Journals (Sweden)

    Debmalya Sanyal

    2012-01-01

    Full Text Available Introduction: Many of the treatment modalities recommended for acromegaly are either too expensive or not available in large parts of India. There is a dearth of treatment and outcome data in Indian patients. Aims and Objectives: The purpose of this study was to analyze the treatment modalities used and the respective outcomes which include remission, recurrence, hypopituitarism, other complications, and mortality. Materials and Methods: This is a retrospective data analysis of 15 acromegaly patients treated at a tertiary care hospital in eastern India. A remission criteria of nadir growth hormone level <1 μg/dl after Oral Glucose tolerance test (OGTT and normal age related IGF-1 levels was used. Results: All patients (100% had macroadenomas. Surgery could not be done in five (33%; three (19.8% refused, two (13.2% had comorbidities. Transsphenoidal surgery (TSS achieved remission in four out of ten (40%. Conventional radiotherapy (CRT failed in all five patients and caused hypopituitarisn in three (60%. Cabergoline (CAB either alone or following surgery achieved remission in one out of four (25% though symptomatic relief and tolerability were remarkable. One patient (7% had pituitary apoplexy with remission, two patients (14.3% died due to CVA. Conclusions: TSS remains the treatment of choice in acromegaly, though in macroadenomas the success is limited. A sizeable proportion of patients refuse or are unfit for surgery. As most of the recommended options are very costly or unavailable, alternative treatment options generally used are CRT or CAB which have limited efficacy. Incidence of hypopituitarism, following CRT is very high.

  8. Pharmacological therapy for acromegaly: a critical review.

    Science.gov (United States)

    Muller, Alex F; Van Der Lely, Aart Jan

    2004-01-01

    The treatment of acromegaly has changed considerably over the last few decades. In the late 1970s, the introduction of the dopamine receptor agonists made it possible to reduce growth hormone (GH) secretion by somatotropinomas for the first time. Thereafter, the introduction of the somatostatin analogues in the early 1980s had major implications. Recently, the first data on the use of genetically engineered human GH receptor (GHR) antagonists that block GH actions have become available. These GHR antagonists reduce both the biochemical abnormalities of acromegaly, as well as improve clinical signs and symptomatology. In this article we firstly review available data on dopamine agonists. Currently these compounds should be considered in patients with a mixed GH-prolactin secreting pituitary adenoma and/or those in whom pre-treatment insulin-like growth factor (IGF)-I concentrations are below 750 microg/L. We then discuss the somatostatin analogues. These compounds are capable of achieving biochemical control of GH and IGF-I in 50-60% of patients and tumour shrinkage in some 30%. In particular, candidates for treatment with these compounds are those patients who have undergone an unsuccessful transsphenoidal operation or who await the therapeutic effect of external pituitary irradiation. In selected patients primary medical therapy with somatostatin analogues is certainly a feasible option. To date, pegvisomant is the only available member of a new class of drugs that was especially designed to block the GHR. Pegvisomant is the most effective treatment for normalising IGF-I concentrations and appears to have a good safety profile. However, liver function tests should be regularly monitored and tumour size should be closely followed. Finally, we propose a treatment algorithm for acromegaly.

  9. Usefulness of an ad hoc questionnaire (Acro-CQ) for the systematic assessment of acromegaly comorbidities at diagnosis and their management at follow-up.

    Science.gov (United States)

    Guaraldi, F; Gori, D; Beccuti, G; Prencipe, N; Giordano, R; Mints, Y; Di Giacomo, V S; Berton, A; Lorente, M; Gasco, V; Ghigo, E; Salvatori, R; Grottoli, S

    2016-11-01

    To determine the validity of a self-administered questionnaire (Acro-CQ) developed to systematically assess the presence, type and time of onset of acromegaly comorbidities. This is a cross-sectional study; 105 acromegaly patients and 147 controls with other types of pituitary adenoma, referred to a specialized Italian Center, autonomously compiled Acro-CQ in an outpatient clinical setting. To test its reliability in a different setting, Acro-CQ was administered via mail to 78 patients with acromegaly and 100 with other pituitary adenomas, referred to a specialized US Center. Data obtained from questionnaires in both settings were compared with medical records (gold standard). Demographics of patients and controls from both countries were similar. In both settings, >95 % of the questionnaires were completely filled; only one item was missed in the others. Concordance with medical record was excellent (k > 0.85) for most of the items, independently from the way of administration, patient age, gender and nationality, pituitary adenoma type and disease activity. Acro-CQ is an inexpensive, highly accepted from patients and reliable tool recommended to expedite systematic collection of relevant clinical data in acromegaly at diagnosis, to be replicated at follow-ups. This tool may guide a targeted, cost-effective management of complications. Moreover, it could be applied to retrieve data for survey studies in both acromegaly and other pituitary adenomas, as information is easily and rapidly accessible for statistical analysis.

  10. Harvey Cushing's ghosts: death and hauntings in modern medicine.

    Science.gov (United States)

    Shin, Paul

    2011-06-01

    The passing of Yale School of Medicine's 2010 Bicentennial occasions a moment of reflecting on the past, present, and future of medical education and research at Yale and beyond. Last June, a ribbon-cutting ceremony inaugurated the opening of the Cushing Center in the Cushing-Whitney Medical Library. Named after Harvey Cushing, an early 20th-century neurosurgeon and former Yale College alum, the dual education/exhibition space now houses hundreds of gross brain specimens constituting the Cushing Tumor Registry. Originally a personal collection, Cushing donated his numerous medical specimens, photographs, and other medical relics from his deathbed, relinquishing the brains to Yale only under the condition that a suitable space be erected to preserve the many specimens. Some 70 years later and after nearly being destroyed, Cushing's wish is fully realized: The once desiccated, hidden brains have been painstakingly restored and are now on view in the Cushing Center. The brains express Cushing's singular and spectral worldview as a surgeon, artist, athlete, soldier, book collector, and historian.

  11. Assessment of cardiac autonomic functions by heart rate recovery, heart rate variability and QT dynamicity parameters in patients with acromegaly.

    Science.gov (United States)

    Dural, Muhammet; Kabakcı, Giray; Cınar, Neşe; Erbaş, Tomris; Canpolat, Uğur; Gürses, Kadri Murat; Tokgözoğlu, Lale; Oto, Ali; Kaya, Ergün Barış; Yorgun, Hikmet; Sahiner, Levent; Dağdelen, Selçuk; Aytemir, Kudret

    2014-04-01

    Cardiovascular complications are the most common causes of morbidity and mortality in acromegaly. However, there is little data regarding cardiac autonomic functions in these patients. Herein, we aimed to investigate several parameters of cardiac autonomic functions in patients with acromegaly compared to healthy subjects. We enrolled 20 newly diagnosed acromegalic patients (55% female, age:45.7 ± 12.6 years) and 32 age- and gender-matched healthy subjects. All participants underwent 24 h Holter recording. Heart rate recovery (HRR) indices were calculated by subtracting 1st, 2nd and 3rd minute heart rates from maximal heart rate. All patients underwent heart rate variability (HRV) and QT dynamicity analysis. Baseline characteristics were similar except diabetes mellitus and hypertension among groups. Mean HRR1 (29.2 ± 12.3 vs 42.6 ± 6.5, p = 0.001), HRR2 (43.5 ± 15.6 vs 61.1 ± 10.8, p = 0.001) and HRR3 (46.4 ± 16.2 vs 65.8 ± 9.8, p = 0.001) values were significantly higher in control group. HRV parameters as, SDNN [standard deviation of all NN intervals] (p = 0.001), SDANN [SD of the 5 min mean RR intervals] (p = 0.001), RMSSD [root square of successive differences in RR interval] (p = 0.001), PNN50 [proportion of differences in successive NN intervals >50 ms] (p = 0.001) and high-frequency [HF] (p = 0.001) were significantly decreased in patients with acromegaly; but low frequency [LF] (p = 0.046) and LF/HF (p = 0.001) were significantly higher in acromegaly patients. QTec (p = 0.009), QTac/RR slope (p = 0.017) and QTec/RR slope (p = 0.01) were significantly higher in patients with acromegaly. Additionally, there were significant negative correlation of disease duration with HRR2, HRR3, SDNN, PNN50, RMSSD, variability index. Our study results suggest that cardiac autonomic functions are impaired in patients with acromegaly. Further large scale studies are needed to exhibit the prognostic significance of impaired autonomic functions in patients with

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

    Science.gov (United States)

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

    2017-07-01

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

  13. Gamma Knife Radiosurgery for Acromegaly

    National Research Council Canada - National Science Library

    Rolston, John D; Blevins, Lewis S

    2012-01-01

    .... 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...

  14. An overview of the epidemiology and genetics of acromegaly.

    Science.gov (United States)

    Daly, A F; Petrossians, P; Beckers, A

    2005-01-01

    Historical data indicate that pituitary tumors represent 10% of intracranial tumors, while adenomas are noted in approximately 14-23% of normal subjects on autopsy or magnetic resonance imaging (MRI). About 2.5% of these tumors stain positive for GH in histopathologic studies. In contrast, the prevalence of clinically diagnosed acromegaly is lower at 36-69 per million population. Ongoing studies indicate that the actual prevalence of acromegaly in the community may be higher than previous epidemiologic data suggest. Acromegaly can occur both sporadically and in the setting of familial conditions, such as multiple endocrine neoplasia type 1 (MEN1) and Carney complex (CNC). Isolated familial somatotropinoma has been described and newer data suggest that acromegaly may also occur in non-MEN1/CNC families in combination with other pituitary tumor phenotypes.

  15. Current and future medical treatments for patients with acromegaly.

    Science.gov (United States)

    Maffezzoni, Filippo; Formenti, Anna Maria; Mazziotti, Gherardo; Frara, Stefano; Giustina, Andrea

    2016-08-01

    Acromegaly is a relatively rare condition of growth hormone (GH) excess associated with significant morbidity and, when left untreated, high mortality. Therapy for acromegaly is targeted at decreasing GH and insulin-like growth hormone 1 levels, ameliorating patients' symptoms and decreasing any local compressive effects of the pituitary adenoma. The therapeutic options for acromegaly include surgery, medical therapies (such as dopamine agonists, somatostatin receptor ligands and the GH receptor antagonist pegvisomant) and radiotherapy. However, despite all these treatments option, approximately 50% of patients are not adequately controlled. In this paper, the authors discuss: 1) efficacy and safety of current medical therapy 2) the efficacy and safety of the new multireceptor-targeted somatostatin ligand pasireotide 3) medical treatments currently under clinical investigation (oral octreotide, ITF2984, ATL1103), and 4) preliminary data on the use of new injectable and transdermal/transmucosal formulations of octreotide. This expert opinion supports the need for new therapeutic agents and modalities for patients with acromegaly.

  16. ontaneous Remission of Acromegaly Due to Apoplexy

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    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

  17. Acromegaly in a non-diabetic cat

    Directory of Open Access Journals (Sweden)

    Federico Fracassi

    2016-04-01

    Full Text Available Case summary A 14-year-old, neutered male European shorthair cat was evaluated for a routine health check. The owner did not report any clinical signs except for respiratory stridor. On physical examination the main findings were broad facial features and increased interdental spaces. On haematology, a mild, non-regenerative anaemia was detected, whereas the serum biochemistry profile and urinalysis were unremarkable. The serum glucose concentration was within the reference interval. Serum insulin-like growth factor-1 concentration was markedly elevated (>1600 ng/ml. The basal serum growth hormone concentration was elevated and decreased only mildly after somatostatin administration. Basal serum insulin concentration was high, and the insulin concentration increased considerably after glucose loading, consistent with insulin resistance. CT scanning of the skull showed an enlarged pituitary gland and increased skull bone thickness. The final diagnosis was acromegaly. Relevance and novel information These findings demonstrate that acromegaly should be pursued and suspected in cats other than those with diabetes mellitus.

  18. The hypertension of Cushing's syndrome: controversies in the pathophysiology and focus on cardiovascular complications.

    Science.gov (United States)

    Isidori, Andrea M; Graziadio, Chiara; Paragliola, Rosa Maria; Cozzolino, Alessia; Ambrogio, Alberto G; Colao, Annamaria; Corsello, Salvatore M; Pivonello, Rosario

    2015-01-01

    Cushing's syndrome is associated with increased mortality, mainly due to cardiovascular complications, which are sustained by the common development of systemic arterial hypertension and metabolic syndrome, which partially persist after the disease remission. Cardiovascular diseases and hypertension associated with endogenous hypercortisolism reveal underexplored peculiarities. The use of exogenous corticosteroids also impacts on hypertension and cardiovascular system, especially after prolonged treatment. The mechanisms involved in the development of hypertension differ, whether glucocorticoid excess is acute or chronic, and the source endogenous or exogenous, introducing inconsistencies among published studies. The pleiotropic effects of glucocorticoids and the overlap of the several regulatory mechanisms controlling blood pressure suggest that a rigorous comparison of in-vivo and in-vitro studies is necessary to draw reliable conclusions. This review, developed during the first 'Altogether to Beat Cushing's syndrome' workshop held in Capri in 2012, evaluates the most important peculiarities of hypertension associated with CS, with a particular focus on its pathophysiology. A critical appraisal of most significant animal and human studies is compared with a systematic review of the few available clinical trials. A special attention is dedicated to the description of the clinical features and cardiovascular damage secondary to glucocorticoid excess. On the basis of the consensus reached during the workshop, a pathophysiology-oriented therapeutic algorithm has been developed and it could serve as a first attempt to rationalize the treatment of hypertension in Cushing's syndrome.

  19. Increased Short-Term Beat-To-Beat Variability of QT Interval in Patients with Acromegaly.

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    Andrea Orosz

    Full Text Available Cardiovascular diseases, including ventricular arrhythmias are responsible for increased mortality in patients with acromegaly. Acromegaly may cause repolarization abnormalities such as QT prolongation and impairment of repolarization reserve enhancing liability to arrhythmia. The aim of this study was to determine the short-term beat-to-beat QT variability in patients with acromegaly. Thirty acromegalic patients (23 women and 7 men, mean age±SD: 55.7±10.4 years were compared with age- and sex-matched volunteers (mean age 51.3±7.6 years. Cardiac repolarization parameters including frequency corrected QT interval, PQ and QRS intervals, duration of terminal part of T waves (Tpeak-Tend and short-term variability of QT interval were evaluated. All acromegalic patients and controls underwent transthoracic echocardiographic examination. Autonomic function was assessed by means of five standard cardiovascular reflex tests. Comparison of the two groups revealed no significant differences in the conventional ECG parameters of repolarization (QT: 401.1±30.6 ms vs 389.3±16.5 ms, corrected QT interval: 430.1±18.6 ms vs 425.6±17.3 ms, QT dispersion: 38.2±13.2 ms vs 36.6±10.2 ms; acromegaly vs control, respectively. However, short-term beat-to-beat QT variability was significantly increased in acromegalic patients (4.23±1.03 ms vs 3.02±0.80, P<0.0001. There were significant differences between the two groups in the echocardiographic dimensions (left ventricular end diastolic diameter: 52.6±5.4 mm vs 48.0±3.9 mm, left ventricular end systolic diameter: 32.3±5.2 mm vs 29.1±4.4 mm, interventricular septum: 11.1±2.2 mm vs 8.8±0.7 mm, posterior wall of left ventricle: 10.8±1.4 mm vs 8.9±0.7 mm, P<0.05, respectively. Short-term beat-to-beat QT variability was elevated in patients with acromegaly in spite of unchanged conventional parameters of ventricular repolarization. This enhanced temporal QT variability may be an early indicator of increased

  20. Active postoperative acromegaly: sustained remission after discontinuation of somatostatin analogues

    Directory of Open Access Journals (Sweden)

    Cristina Alvarez-Escola

    2016-11-01

    Full Text Available In patients with active acromegaly after pituitary surgery, somatostatin analogues are effective in controlling the disease and can even be curative in some cases. After treatment discontinuation, the likelihood of disease recurrence is high. However, a small subset of patients remains symptom-free after discontinuation, with normalized growth hormone (GH and insulin-like growth factor (IGF1 levels. The characteristics of patients most likely to achieve sustained remission after treatment discontinuation are not well understood, although limited evidence suggests that sustained remission is more likely in patients with lower GH and IGF1 levels before treatment withdrawal, in those who respond well to low-dose treatment, in those without evidence of adenoma on an MRI scan and/or in patients who receive long-term treatment. In this report, we describe the case of a 56-year-old female patient treated with lanreotide Autogel for 11 years. Treatment was successfully discontinued, and the patient is currently disease-free on all relevant parameters (clinical, biochemical and tumour status. The successful outcome in this case adds to the small body of literature suggesting that some well-selected patients who receive long-term treatment with somatostatin analogues may achieve sustained remission.

  1. A practical approach to acromegaly management in Latin America

    OpenAIRE

    Bronstein, Marcello D.; Oscar D. Bruno; Abreu, Alin; Mangupli, Ruth; Mercado, Moisés

    2013-01-01

    Introduction Evidence-based treatment guidelines have undoubtedly advanced medical practice and supported optimal management of acromegaly, but their application may be hampered by limited access to the latest treatment options. Methods In this retrospective, narrative review, the authors revisited existing treatment guidelines for acromegaly in Latin America. These were considered in conjunction with published evidence chosen at the authors’ discretion. Findings In a socially and economicall...

  2. Prevalence of cerebral aneurysm in patients with acromegaly.

    Science.gov (United States)

    Oshino, Satoru; Nishino, Akio; Suzuki, Tsuyoshi; Arita, Hideyuki; Tateishi, Akihiro; Matsumoto, Katsumi; Shimokawa, Toshio; Kinoshita, Manabu; Yoshimine, Toshiki; Saitoh, Youichi

    2013-06-01

    The prevalence of cerebral aneurysm was retrospectively investigated in 208 patients with acromegaly relative to the rate of cerebral aneurysm in a group of control subjects. Neuroradiological examinations of the cerebral vascular system were conducted in 208 acromegaly patients (101 men; mean age, 48.8 years). The prevalence of cerebral aneurysm in the acromegaly patients was compared to that in a control group consisting of 7,390 subjects who underwent "brain checkup" between 2006 and 2008 (mean age, 51.6 years). In the acromegaly group, cerebral aneurysm was detected in 4.3 % of patients. By sex, the prevalence was 6.9 % in males, a significantly proportion than that in the control group with an odds ratio of 4.40. The prevalence in females did not differ between the two groups. In the acromegaly group, the rate of hypertension was significantly higher in the patients with aneurysm compared to those without aneurysm. Multiple logistic regression identified acromegaly as a significant factor related to the prevalence of cerebral aneurysm in all male subjects; other factors, such as age, hypertension and smoking, were not found to be significant. A significantly higher prevalence of cerebral aneurysm was detected in male patients with acromegaly. This finding indicates that excess growth hormone or insulin-like growth factor 1 affects the cerebral vascular wall, resulting in aneurysm formation. In addition to known systematic complications in the cardiovascular, respiratory, metabolic, and other systems, the risk of cerebral aneurysm should be considered in the management of acromegaly.

  3. Diagnostic delay is associated with psychosocial impairment in acromegaly.

    Science.gov (United States)

    Siegel, Sonja; Streetz-van der Werf, Christine; Schott, Jennifer S; Nolte, Kay; Karges, Wolfram; Kreitschmann-Andermahr, Ilonka

    2013-12-01

    The aim of this study was to systematically assess health care utilisation, diagnostic delay and psychosocial impairment in patients with acromegaly in rural versus urban health care environments. 41 patients with acromegaly were questioned to time lapse of symptom onset, first seeking medical advice and time of acromegaly diagnosis. Quality of life (QoL), and psychosocial impairment (depression, daytime sleepiness, sleep disturbances, disturbances of body image) were measured by self-assessment questionnaires. Patients were grouped into living in rural health care environments (RHCE, n = 22 patients) or urban health care environments (UHCE, n = 19 patients) using data on population density from the German Federal Statistical Office. RHCE patients waited significantly longer (2.5 vs. 0.89 years; p = .025) after symptom onset before seeking medical advice, but diagnosis of acromegaly was established at least as quickly as in UHCE (1.45 vs. 2.74 years; n.s.). There was a consistent trend toward more psychosocial impairment in UHCE which reached significance for sleep disturbances (p = .004). For all patients significant correlations between time delay of diagnostic process (defined as first visit to the doctor because of acromegaly-related symptoms and establishment of acromegaly diagnosis) and psychological QoL, depression, daytime sleepiness, sleep disorders and body image emerged. Patients with acromegaly in UHCE experienced more psychosocial impairment than patients in RHCE. The correlation of significantly increased psychosocial impairment and delay of diagnosis by the physician may reflect long-lasting embitterment in patients with acromegaly and should be considered during psychosocial counselling.

  4. Fractionated stereotactic radiotherapy in patients with acromegaly: an interim single-centre audit

    DEFF Research Database (Denmark)

    Roug, Anne Stidsholt; Rasmussen, Åse Krogh; Juhler, M;

    2010-01-01

    To evaluate the effect of fractionated stereotactic radiotherapy (FSRT) in acromegaly in a retrospective analysis.......To evaluate the effect of fractionated stereotactic radiotherapy (FSRT) in acromegaly in a retrospective analysis....

  5. Late presentation of acromegaly in medically controlled prolactinoma patients

    Science.gov (United States)

    Manuylova, Ekaterina; Calvi, Laura M; Hastings, Catherine; Vates, G Edward; Johnson, Mahlon D; Cave, William T

    2016-01-01

    Summary Co-secretion of growth hormone (GH) and prolactin (PRL) from a single pituitary adenoma is common. In fact, up to 25% of patients with acromegaly may have PRL co-secretion. The prevalence of acromegaly among patients with a newly diagnosed prolactinoma is unknown. Given the possibility of mixed GH and PRL co-secretion, the current recommendation is to obtain an insulin-like growth factor-1 (IGF-1) in patients with prolactinoma at the initial diagnosis. Long-term follow-up of IGF-1 is not routinely done. Here, we report two cases of well-controlled prolactinoma on dopamine agonists with the development of acromegaly 10–20 years after the initial diagnoses. In both patients, a mixed PRL/GH-cosecreting adenoma was confirmed on the pathology examination after transsphenoidal surgery (TSS). Therefore, periodic routine measurements of IGF-1 should be considered regardless of the duration and biochemical control of prolactinoma. Learning points: Acromegaly can develop in patients with well-controlled prolactinoma on dopamine agonists. The interval between prolactinoma and acromegaly diagnoses can be several decades. Periodic screening of patients with prolactinoma for growth hormone excess should be considered and can 
lead to an early diagnosis of acromegaly before the development of complications. PMID:27855229

  6. Health outcomes in acromegaly: depression and anxiety are promising targets for improving reduced quality of life

    Directory of Open Access Journals (Sweden)

    Victor Jacobus Geraedts

    2015-01-01

    Full Text Available IINTRODUCTION. Remission criteria of acromegaly are based on biochemical variables, i.e. normalization of increased hormone levels. However, the established reduction in Quality of Life (QoL is suggested to be independent of biochemical control. The aim of this study was to test which aspects predict Qol best in acromegaly. METHODS/Design. This is a prospective cohort study in 80 acromegalic patients, with a cross-sectional and longitudinal part. The main outcome measure was health-related quality of life (QoL, measured by a generic and a disease-specific questionnaire (the SF-36 and AcroQol. Main predictors were age, gender, biochemical control, disease characteristics, treatment modalities and psychopathology. RESULTS. Our cohort of 80 acromegalics had a mean age 54.7 ± 12.3 years with an average disease duration of 10.8 ± 10.0 years. Ratio macro-/microadenoma was 54/26. In adjusted mixed method models, we found that psychopathology significantly predicts QoL in acromegaly (in models including the variables age, gender, disease duration, tumor size, basal hormone levels, relevant treatment modalities and relevant comorbidities, with a higher degree of psychopathology indicating a lower QoL (depression vs. AcroQoL: B=-1.175, p<0.001, depression vs. SF36: B=-1.648, p<0.001, anxiety vs. AcroQoL: B=-0.399, p<0.001, anxiety vs. SF36: B=-0.661, p<0.001. The explained variances demonstrate superiority of psychopathology over biochemical control and other variables in predicting QoL in our models. DISCUSSION. Superiority of psychopathology over biochemical control calls for a more extensive approach regarding diagnosing depression and anxiety in pituitary adenomas to improve QoL. Depressive symptoms and anxiety are modifiable factors that might provide valuable targets for possible future treatment interventions.

  7. Tumorigenesis of Papillary Thyroid Cancer Is Not BRAF-Dependent in Patients with Acromegaly

    OpenAIRE

    Hee Kyung Kim; Ji Shin Lee; Min Ho Park; Jin Seong Cho; Jee Hee Yoon; Soo Jeong Kim; Ho-Cheol Kang

    2014-01-01

    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 BRAF V600E 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...

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

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    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

  9. Cushing's syndrome masquerading as treatment resistant depression

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    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.

  10. [Active acromegaly and gigantism: some clinical characteristics of 50 patients].

    Science.gov (United States)

    Pumarino, H; Oviedo, S; Michelsen, H; Campino, C

    1991-08-01

    50 patients with autonomous growth hormone excess (48 with adult acromegaly and 2 with gigantism) were studied between 1966 to 1986 (2.38 pts/year). Characteristic clinical presentation, an increase in growth hormone (GH) uninhibited by glucose, and/or hyperphosphemia and hyperhydroxiprolinuria were present in all patients. No cases of hypercalcemia were recorded. Phosphemia was increased in 55.8%, alkaline phosphatases in 61.7%, calciuria in 26.9% and hydroxyprolinuria in 74.2% of the patients. Basal GH was over 5 ng/ml (89.9 DS +/- 170.9) in 42 pts, and in 37 was not suppressed after glucose administration, 38% had an increased (paradoxical response) and 62% a flat response (less than 50% change of basal values). TRH test was performed in 14 patients, 8 presented an increase in GH titer. Hyperprolactinemia was seen in 4 of 12 patients in whom this hormone was measured. The size of the sella turcica was increased in 93%, and although the larger sellar size correlated to higher levels of GH, correlation was not significant. 20% of the pts had rheumatological disease, 14% goiter, 12% cardiac disease, 26.5% had diastolic hypertension and 4% renal lithiasis (hypercalciuric pts). 38% had hyperglycemia with a diabetic glucose tolerance test and 18% had non-diabetic abnormal glucose tolerance test.

  11. Acromegaly: Role of Surgery in the Therapeutic Armamentarium

    Directory of Open Access Journals (Sweden)

    Gerardo Guinto

    2012-01-01

    Full Text Available Acromegaly is a complex disease that requires the intervention of a multidisciplinary team. The most frequent clinical manifestations are growing of distal parts of the body and some areas of the face. Patients may also present arterial hypertension, diabetes mellitus, colonic polyps, cardiomegaly, neurological and endocrine changes secondary to the presence of a GH-secreting tumor in pituitary or extrapituitary origin, or eutopic hypothalamic GHRH hypersecretion and peripheral GHRH hypersecretion. Surgery is the first treatment used for most patients, regardless of the cause. In the great majority of cases, pituitary tumor can be removed through a transsphenoidal approach. Craniotomy is reserved for those cases with giant tumors, particularly when they grow toward the middle or posterior cranial fossa. Best surgical results are obtained when the tumor is confined into the sella turcica or if it has a regular suprasellar extension. When the disease cannot be controlled with surgery, medical treatment is indicated. Somatostatin analogues are included as the first line of medication, followed by dopamine agonist and growth hormone receptors antagonists. Radiation therapy can be also indicated in two main forms for residual tumor with medically refractory patients: radiosurgery for small tumors or fractionated stereotactic radiotherapy for larger ones.

  12. Quality of life is impaired in association with the need for prolonged postoperative therapy by somatostatin analogs in patients with acromegaly

    NARCIS (Netherlands)

    Postma, M.R.; Netea-Maier, R.T.; van den Berg, G.; Homan, J.; Sluiter, W.J.; Wagenmakers, M.A.; van den Bergh, A.C.M.; Wolffenbuttel, B.H.R.; Hermus, A.R.M.M.; van Beek, A.P.

    Objective: To assess the influence of long-acting somatostatin analogs (SSTA) after initial pituitary surgery on long-term health-related quality of life (HR-QoL) in relation to disease control in patients with acromegaly. Design: This is a cross-sectional study in two tertiary referral centers in

  13. Quality of life is impaired in association with the need for prolonged postoperative therapy by somatostatin analogs in patients with acromegaly.

    NARCIS (Netherlands)

    Postma, M.R.; Netea-Maier, R.T.; Berg, G. van den; Homan, J.; Sluiter, W.J.; Wagenmakers, M.A.E.M.; Bergh, A.C. van den; Wolffenbuttel, B.H.R.; Hermus, A.R.M.M.; Beek, A.P. van

    2012-01-01

    OBJECTIVE: To assess the influence of long-acting somatostatin analogs (SSTA) after initial pituitary surgery on long-term health-related quality of life (HR-QoL) in relation to disease control in patients with acromegaly. DESIGN: This is a cross-sectional study in two tertiary referral centers in