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Sample records for acromegaly

  1. Acromegaly

    Chanson Philippe

    2008-06-01

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

  2. [Acromegaly: current view].

    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.

  3. The History of Acromegaly.

    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.

  4. ACROMEGALY: A CASE REPORT

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    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...

  5. Growth hormone, growth factors, and acromegaly

    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.

  6. Medical therapy in acromegaly.

    Sherlock, Mark

    2011-05-01

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

  7. Cardiovascular complications in acromegaly.

    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.

  8. Medical Therapy of Acromegaly

    U. Plöckinger

    2012-01-01

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

  9. ACROMEGALY: A CASE REPORT

    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.

  10. Acromegaly: Beyond surgery

    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 ...

  11. Diabetic retinopathy in acromegaly

    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...

  12. Acromegaly associated with gangliocytoma.

    Crowley, R K

    2009-09-30

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

  13. Acromegaly : irreversible clinical consequences

    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

  14. Ventricular tachycardia in acromegaly.

    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.

  15. [Drug therapy for acromegaly].

    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.

  16. Radiation techniques for acromegaly

    Minniti Giuseppe

    2011-12-01

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

  17. Acromegaly: Beyond surgery

    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.

  18. Acromegaly: Beyond surgery.

    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.

  19. Diabetic retinopathy in acromegaly

    Said Azzoug

    2014-01-01

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

  20. Sleep apnea in active acromegaly.

    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.

  1. Macroglossia in acromegaly and hypothyroidism.

    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.

  2. Gamma Knife Radiosurgery for Acromegaly

    John D. Rolston

    2012-01-01

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

  3. [Cardiological findings in acromegaly].

    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

  4. Diabetic ketoacidosis in a patient with acromegaly

    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

  5. A case of acromegaly.

    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.

  6. Neuromuscular complications of acromegaly.

    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.

  7. Epidemiology of acromegaly.

    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.

  8. Treatment Factors That Influence Mortality in Acromegaly.

    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. Carpal tunnel syndrome and acromegaly.

    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.

  10. Radiation therapy of acromegaly.

    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

  11. Neurosurgical Treatment of Acromegaly.

    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.

  12. Cutaneous Findings in Patients with Acromegaly

    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...

  13. Clinical Manifestations and Diagnosis of Acromegaly

    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...

  14. Megaduodenum in a patient with acromegaly

    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...

  15. Pasireotide in Acromegaly: A Review.

    McKeage, Kate

    2015-06-01

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

  16. Sleep-disordered breathing in acromegaly

    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.

  17. [Epidemiology of acromegaly in Spain].

    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.

  18. Growth hormone deficiency in treated acromegaly.

    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.

  19. Development of acromegaly in patients with prolactinomas

    Andersen, Marianne; Hagen, Casper; Frystyk, Jan

    2003-01-01

    OBJECTIVES: Patients with prolactinomas and patients with acromegaly often have heterogenous adenomas. In this study we have focused on patients with prolactinomas who developed acromegaly and acromegalic patients with hyperprolactinaemia. Our hypothesis is that some patients with 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. Clinical Manifestations and Diagnosis of Acromegaly

    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.

  1. Bone and Joint Disorders in Acromegaly.

    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.

  2. Megaduodenum in a patient with acromegaly

    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.

  3. Cardiovascular aspects in acromegaly: effects of treatment.

    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.

  4. Cutaneous findings in patients with acromegaly.

    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.

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

    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...

  6. Characteristics of Acromegaly in Korea with a Literature Review

    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...

  7. Prevalence of diabetes mellitus in patients with acromegaly

    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

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

    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.

  9. Cardiovascular complications in acromegaly: methods of assessment.

    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.

  10. Multiple Myeloma in a Patient with Acromegaly

    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.

  11. Acromegaly

    ... 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. Acromegaly

    ... 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 ...

  13. Unsuccessful treatment of acromegaly with medroxyprogesterone acetate.

    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.

  14. Urinary growth hormone excretion in acromegaly

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

    1993-01-01

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

  15. 60 YEARS OF NEUROENDOCRINOLOGY: Acromegaly.

    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.

  16. Acromegaly without acral changes: A rare presentation

    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.

  17. An update on the treatment of acromegaly

    Edling KL

    2013-02-01

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

  18. Active acromegaly enhances spontaneous parathyroid hormone pulsatility.

    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.

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

    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.

  20. Acromegaly incidence, prevalence, complications and long-term prognosis

    Dal, Jakob; Feldt-Rasmussen, Ulla; Andersen, Marianne;

    2016-01-01

    DESIGN: Valid data on acromegaly incidence, complications and mortality are scarce. The Danish Health Care System enables nationwide studies with complete follow-up and linkage among health-related databases to assess acromegaly incidence, prevalence, complications and mortality in a population......-based cohort study. METHOD: All incident cases of acromegaly in Denmark (1991-2010) were identified from health registries and validated by chart review. We estimated the annual incidence rate of acromegaly per 10(6) person-years (py) with 95% confidence intervals (95% CIs). For every patient, 10 persons were...... (HR: 2.1 (95% CI: 1.6-2.6)). The complication risk was also increased before the diagnosis of acromegaly. Overall mortality risk was elevated (HR: 1.3 (95% CI: 1.0-1.7)) but uninfluenced by treatment modality. CONCLUSION: (i) The incidence rate and age at diagnosis of acromegaly have been stable over...

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

    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.

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

    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, ...

  3. Prevalence of diabetes mellitus in patients with acromegaly.

    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.

  4. MRI of pituitary adenomas in acromegaly

    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. Acromegaly said to respond to proton therapy

    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.

  6. Treatment of acromegaly by external irradiation

    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).

  7. Cushing, acromegaly, GH deficiency and tendons

    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...

  8. Glioblastoma multiforme after radiotherapy for acromegaly

    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.

  9. [Carcinoma of the hypophysis with acromegaly].

    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.

  10. [Myopathy in acromegaly. Report of two cases].

    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)

  11. A consensus on criteria for cure of acromegaly

    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

  12. Combined treatment of somatostatin analogues with pegvisomant in acromegaly

    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

  13. Depression in acromegaly treated with escitalopram and cognitive therapy

    De Sousa Avinash

    2009-01-01

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

  14. [Current diagnosis and treatment of acromegaly].

    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.

  15. Elevated circulating somatostatin levels in acromegaly.

    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.

  16. Combination of Klinefelter Syndrome and Acromegaly

    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

  17. The Role of Radiotherapy in Acromegaly.

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

    2016-01-01

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

  18. Current pharmacotherapy for acromegaly: a review.

    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.

  19. QT dispersion in patients with acromegaly.

    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.

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

    Juan Francisco Martín-Rodríguez

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

  1. Investigation of the Vitamin D Receptor Polymorphisms in Acromegaly Patients

    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. Investigation of the Vitamin D Receptor Polymorphisms in Acromegaly Patients

    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

  3. Outcome in acromegaly: A retrospective analysis

    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.

  4. Pharmacological therapy for acromegaly: a critical review.

    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.

  5. Association of Acromegaly and Multiple Myeloma: A Case Report

    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

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

    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.

  7. ontaneous Remission of Acromegaly Due to Apoplexy

    Cevdet Duran

    2008-01-01

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

  8. Acromegaly in a non-diabetic cat

    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.

  9. An overview of the epidemiology and genetics of acromegaly.

    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.

  10. Prevalence of cerebral aneurysm in patients with acromegaly.

    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.

  11. A practical approach to acromegaly management in Latin America

    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...

  12. Diagnostic delay is associated with psychosocial impairment in acromegaly.

    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.

  13. Late presentation of acromegaly in medically controlled prolactinoma patients

    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

  14. Fractionated stereotactic radiotherapy in patients with acromegaly: an interim single-centre audit

    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....

  15. Tumorigenesis of Papillary Thyroid Cancer Is Not BRAF-Dependent in Patients with Acromegaly

    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...

  16. Patients' experience of nursing with Acromegaly

    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...

  17. Subclinical carpal tunnel syndrome in acromegaly.

    Kameyama, S; Tanaka, R; Hasegawa, A; Tamura, T; Kuroki, M

    1993-08-01

    Median nerve conduction was studied in 16 acromegalic patients with asymptomatic carpal tunnel syndrome (CTS) to examine the incidence of subclinical CTS. Thirteen patients (81%) and 23 hands (72%) demonstrated subclinical CTS, 10 bilaterally and three unilaterally in the dominant hand. The incidence reflects the greater sensitivity of the inching method for detecting focal conduction abnormalities. Two of three patients without subclinical CTS showed normal plasma somatomedin-C concentration despite growth hormone hypersecretion. Following adenomectomy, nerve conduction normalized in only six hands (26%). The postoperative persistence of the conduction delay implies that irreversible narrowing of the carpal tunnel rather than reversible soft tissue edema is the principal cause of CTS associated with acromegaly.

  18. Gene Expression Signature in Adipose Tissue of Acromegaly Patients.

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

    2015-01-01

    To study the effect of chronic excess growth hormone on adipose tissue, we performed RNA sequencing in adipose tissue biopsies from patients with acromegaly (n = 7) or non-functioning pituitary adenomas (n = 11). The patients underwent clinical and metabolic profiling including assessment of HOMA-IR. Explants of adipose tissue were assayed ex vivo for lipolysis and ceramide levels. Patients with acromegaly had higher glucose, higher insulin levels and higher HOMA-IR score. We observed several previously reported transcriptional changes (IGF1, IGFBP3, CISH, SOCS2) that are known to be induced by GH/IGF-1 in liver but are also induced in adipose tissue. We also identified several novel transcriptional changes, some of which may be important for GH/IGF responses (PTPN3 and PTPN4) and the effects of acromegaly on growth and proliferation. Several differentially expressed transcripts may be important in GH/IGF-1-induced metabolic changes. Specifically, induction of LPL, ABHD5, and NRIP1 can contribute to enhanced lipolysis and may explain the elevated adipose tissue lipolysis in acromegalic patients. Higher expression of TCF7L2 and the fatty acid desaturases FADS1, FADS2 and SCD could contribute to insulin resistance. Ceramides were not different between the two groups. In summary, we have identified the acromegaly gene expression signature in human adipose tissue. The significance of altered expression of specific transcripts will enhance our understanding of the metabolic and proliferative changes associated with acromegaly.

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

    P Dutta

    2012-01-01

    Full Text Available 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 were based on standard criteria. Results:Out of 150 patients with acromegaly, 6 patients had overt CHF (4.0%, of which 4 presented with the features of CHF and 2 developed during the course of illness. Three patients had hypertension and 1 had diabetes. Baseline echocardiography showed severe biventricular dysfunction and global hypokinesia in all. Angiography showed dilated hypokinetic left ventricle with normal coronaries in 3, it was confirmed at autopsy in 1. Three underwent trans-sphenoidal surgery, 1 received somatostatin analogue as primary treatment modality. Normalization of growth hormone and IGF-1 led to improvement in cardiac function in 1, 1 patient lost to follow up, and 4 died during the course of illness. In 1 patient, autopsy was performed and cardiac specimen revealed normal coronaries, concentric ventricular hypertrophy, and dilatation with myofibrolysis and interfascicular fibrosis. Conclusion:Prevalence of overt CHF is 4% in present series. Overt CHF carries poor prognosis and hence, this complication should be recognized at earliest, and medical management to normalized cardiac function should be given utmost priority.

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

    Hüsniye Başer

    2014-06-01

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

  1. Restrictive extraocular myopathy: A presenting feature of acromegaly

    Steven Heireman

    2011-01-01

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

  2. Malignant ventricular tachycardia in acromegaly: a case report

    Zhe An

    Full Text Available CONTEXT: In patients with acromegaly, cardiovascular complications are the main cause of death; sudden death has been associated with ventricular tachyarrhythmias. In other patients with life-threatening malignant ventricular tachyarrhythmias, surgical placement of an implantable cardioverter-defibrillator (ICD has proved highly effective in reducing sudden death rates. CASE REPORT: The present article reports the case of a 50-year-old male acromegalic patient who presented symptoms of syncope induced by ventricular tachycardia. An ICD was surgically implanted and a pituitary adenoma, which was responsible for the acromegaly, was completely removed in the same procedure. The surgery was successful and the ventricular arrhythmias were effectively terminated. During six months of follow-up, no documented arrhythmic episodes occurred. CONCLUSION: In patients with acromegaly, malignant ventricular tachyarrhythmia might be effectively controlled by implantation of an ICD and surgical removal of the pituitary adenoma.

  3. Late presentation of acromegaly in medically controlled prolactinoma patients

    Ekaterina Manuylova

    2016-10-01

    Full Text Available 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.

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

    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.

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

    Proff Peter

    2009-08-01

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

  6. GH-producing mammary tumors in two dogs with acromegaly.

    Murai, Atsuko; Nishii, Naohito; Morita, Takehito; Yuki, Masashi

    2012-06-01

    Two intact female dogs were admitted for growing mammary tumors. They had symptoms of acromegaly including weight gain, enlargement of the head, excessive skin folds, and inspiratory stridor. Serum concentrations of growth hormone (GH), insulin-like growth factor-I (IGF-I), and insulin were elevated in the two cases. From these findings, both dogs were diagnosed with acromegaly. In case 1, the GH, IGF-I, and insulin levels subsided after removal of the focal benign mammary tumors and ovariohysterectomy. In case 2, those levels subsided after removal of only focal mammary carcinoma. In both cases, immunohistochemical investigations for GH were positive in the mammary tumor cells but not in the normal mammary glands. We concluded that GH-producing mammary tumors caused the present acromegaly.

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

    Cuevas-Ramos, Daniel; Fleseriu, Maria

    2016-01-01

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

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

    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.

  9. Investigation of the Vitamin D Receptor Polymorphisms in Acromegaly Patients

    Muzaffer Ilhan; Bahar Toptas-Hekimoglu; Ilhan Yaylim; Seda Turgut; Saime Turan; Ozcan Karaman; Ertugrul Tasan

    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...

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

    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.

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

    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...

  12. Growth Hormone and Insulin Signaling in Acromegaly

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

    2016-01-01

    CONTEXT: Somatostatin analogues (SA) used in acromegaly to suppress GH secretion and tumor growth also suppress insulin secretion and may impact GH signaling. OBJECTIVE: To compare GH and insulin signaling after intravenous GH exposure in acromegalic patients controlled by surgery (n=9) or SA (n=9...... MEASURES: GH and insulin signalling in muscle and fat. GH and IGF-I in serum and interstitial fluid; insulin and FFA in serum. RESULTS: The groups were comparable as regards GH and IGF-I. The SA group exhibited higher FFA and glucose levels; basal SOCS1 mRNA in fat was increased in the SA group...... and correlated positively with SA dose (r(2)= 0.54, P=0.04). GH-induced GH signalling (pSTAT5b) in muscle occurred in both groups together with increased expression of SOCS and CISH genes. GH-induced pAKTthr(308) was observed in SA patients. In both groups mRNA expression of PTEN, a suppressor of insulin...

  13. Results of Gamma Knife Radiosurgery in Acromegaly

    Alberto Franzin

    2012-01-01

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

  14. Herniation of cerebellar tonsils in acromegaly: prevalence, pathogenesis and clinical impact.

    Manara, Renzo; Bommarito, Giulia; Rizzati, Silvia; Briani, Chiara; Della Puppa, Alessandro; Citton, Valentina; Zanchetta, Eva; Zerbo, Fabio; Ermani, Mario; Martini, Chiara; Mantero, Franco; Sicolo, Nicola; Maffei, Pietro; Scaroni, Carla

    2013-03-01

    Herniation of cerebellar tonsils (CTH) might occur in acromegaly patients and improve after acromegaly treatment. Our study investigated CTH prevalence in acromegaly, its relationship with clinical, laboratory and neuroimaging findings and its possible pathogenesis and clinical impact. 150 acromegaly patients (median-age 56 years, age-range 21-88, 83 females) underwent brain magnetic resonance imaging (MRI). Clinical data, laboratory and pituitary adenoma imaging findings were recorded. CTH, posterior cranial fossa area, tentorial angle, clivus, supraocciput and Twining's line length were measured in acromegaly patients and controls, who included MRI of 115 consecutive subjects with headache or transient neurological deficits (control group-1) and 24 symptomatic classic Chiari 1 malformation patients (control group-2). Acromegaly patients were interviewed for symptoms known to be related with CTH. 22/150 acromegaly patients (15 %) and 8/115 control group-1 subjects presented with CTH (p = 0.04). In acromegaly patients, CTH correlated positively with younger age and inversely with GH-receptor antagonist treatment. Control group-2 had a shorter clivus than CTH acromegaly patients (40.4 ± 3.2 mm vs 42.5 ± 3.3 mm, p < 0.05), while posterior fossa measures did not differ among acromegaly subgroups (with and without CTH) and control group-1. Headache and vision problems were more frequent in CTH acromegaly patients (p < 0.05); two acromegaly patients presented with imaging and neurological signs of syringomyelia. Despite no signs of posterior fossa underdevelopment or cranial constriction, CTH is more frequent in acromegaly patients and seems to contribute to some disabling neurological symptoms.

  15. Current status and future directions of pharmacological therapy for acromegaly.

    Mercado, Moisés; Espinosa, Etual; Ramírez, Claudia

    2016-09-01

    Acromegaly is a chronic systemic disorder caused in the vast majority of cases by a GH-secreting pituitary adenoma and resulting in significant morbidity and mortality if left untreated. The treatment of choice is the trans-sphenoidal resection of the adenoma, and although 80% of patients with microadenomas or confined macroadenomas achieve biochemical remission, the surgical success rate for patients harboring tumors with extrasellar extension is below 50%. Thus, a considerable proportion of patients will require some form of adjuvant treatment. Acromegaly can be approached pharmacologically by inhibiting GH secretion by the tumor (somatostatin analogues, dopamine agonists) or by antagonizing GH actions at its target tissues (GH receptor antagonists). The primary pharmacological treatment of acromegaly is increasingly gaining acceptance by both physicians and patients. The decision to use primary pharmacological treatment has to take into account the clinical characteristics of the patient (presence of comorbidities that significantly increase the surgical risk) and the biological nature of the adenoma (tumor size and location), as well as other aspects such as the availability of a pituitary surgeon and the cost of medications. This review provides a critical summary and update of the pharmacological treatment of acromegaly focusing both, on well-established agents and strategies as well as on novel compounds that are currently being developed.

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

    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.

  17. Zollinger-Ellison syndrome, acromegaly, and colorectal neoplasia

    Tobi, M; Cats, A; Maliakkal, BJ; Kinzie, JL; Maliakkal, R; Dullaart, RPF; Luk, GD

    1997-01-01

    Zollinger-Ellison syndrome (ZES) and acromegaly are two hypersecretory states in which colorectal neoplasia has been described, but the incidence in the former condition may not be increased. We describe four patients with colorectal neoplasia associated with the ZES and review other published cases

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

    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. Stimulation of growth hormone by vasoactive intestinal polypeptide in acromegaly.

    Chihara, K; Kaji, H; Minamitani, N; Kodama, H; Kita, T; Goto, B; Chiba, T; Coy, D H; Fujita, T

    1984-01-01

    Vasoactive intestinal polypeptide (VIP) was administered as an iv bolus of 1 micrograms/kg BW to 8 acromegalic patients and in doses of 0.5 and 1 microgram/kg BW to 15 normal volunteers. Both systolic and diastolic blood pressures decreased, and pulse rate increased transiently after VIP injection. VIP stimulated PRL release from the anterior pituitary in normal subjects. Plasma PRL responses to VIP in women were dose dependent and larger than those in men. On the other hand, plasma GH levels rose markedly after VIP injection in all 6 patients with untreated acromegaly. In 2 patients studied after transsphenoidal microadenomectomy, there was no plasma GH response to VIP. In 2 other patients with inactive acromegaly as well as in normal subjects, VIP failed to affect plasma GH levels. In all 6 patients with active acromegaly, LRH (1-2 micrograms/kg BW, iv) did not increase plasma GH levels, but TRH (5-10 micrograms/kg BW, iv) caused significant increases in plasma GH, the magnitude of which was not similar to that of increases seen after VIP injection. Paradoxical GH responses to TRH were not observed in patients in the inactive phase after transsphenoidal surgery. These findings suggest that VIP stimulates GH release in vivo in acromegalic patients. A VIP test as well as a TRH test offer promise as simple and reliable techniques to evaluate the activity of acromegaly, particularly after transsphenoidal surgery.

  20. Pegvisomant and cabergoline combination therapy in acromegaly.

    Bernabeu, I; Alvarez-Escolá, C; Paniagua, A E; Lucas, T; Pavón, I; Cabezas-Agrícola, J M; Casanueva, F F; Marazuela, M

    2013-03-01

    Combination with cabergoline may offer additional benefits to acromegalic patients on pegvisomant monotherapy. We evaluated the safety and efficacy profile of this combination and investigated the determinants of response. An observational, retrospective, cross-sectional study. Fourteen acromegalic patients (9 females), who were partially resistant to somatostatin analogs and on pegvisomant monotherapy. Cabergoline was added because of the presence of persistent mildly increased IGF-I. The mean follow-up time was 18.3 ± 10.4 months. The efficacy and safety profile was assessed. The influence of clinical and biochemical characteristics on treatment efficacy was studied. IGF-I levels returned to normal in 4 patients (28%) at the end of the study. In addition, some decline in IGF-I levels was observed in a further 5 patients. The % IGF-I decreased from 158 ± 64% to 124 ± 44% (p = 0.001). The average change in IGF-I was -18 ± 27% (range -67 to +24%). Lower baseline IGF-I (p = 0.007), female gender (p = 0.013), lower body weight (p = 0.031), and higher prolactin (PRL) levels (p = 0.007) were associated with a better response to combination therapy. There were no significant severe adverse events. Significant tumour shrinkage was observed in 1 patient. Combination therapy with pegvisomant and cabergoline could provide better control of IGF-I in some patients with acromegaly. Baseline IGF-I levels, female gender, body weight, and PRL levels affect the response to this combination therapy.

  1. Expert consensus document: A consensus on the medical treatment of acromegaly.

    Giustina, Andrea; Chanson, Philippe; Kleinberg, David; Bronstein, Marcello D; Clemmons, David R; Klibanski, Anne; van der Lely, Aart J; Strasburger, Christian J; Lamberts, Steven W; Ho, Ken K Y; Casanueva, Felipe F; Melmed, Shlomo

    2014-04-01

    In March 2013, the Acromegaly Consensus Group met to revise and update guidelines for the medical treatment of acromegaly. The meeting comprised experts skilled in the medical management of acromegaly. The group considered treatment goals covering biochemical, clinical and tumour volume outcomes, and the place in guidelines of somatostatin receptor ligands, growth hormone receptor antagonists and dopamine agonists, and alternative modalities for treatment including combination therapy and novel treatments. This document represents the conclusions of the workshop consensus.

  2. Effects of previous growth hormone excess and current medical treatment for acromegaly on cognition

    Brummelman, Pauline; Koerts, Janneke; Dullaart, Robin P. F.; van den Berg, Gerrit; Tucha, Oliver; Wolffenbuttel, Bruce H R; van Beek, Andre P.

    2012-01-01

    Background In untreated acromegaly patients, decreased cognitive functioning is reported to be associated with the degree of growth hormone (GH) and IGF-1 excess. Whether previous GH excess or current medical treatment for acromegaly specifically affects cognition remains unclear. The aim of this study was to compare cognitive functioning of patients who are treated for acromegaly with patients with non-functioning pituitary adenomas (NFA). In addition, we assessed the influence of prolonged ...

  3. Incidence and prevalence of acromegaly in a large US health plan database

    Burton, Tanya; Le Nestour, Elisabeth; Neary, Maureen; Ludlam, William H.

    2016-01-01

    Purpose Incidence and prevalence estimates of acromegaly in the United States (US) are limited. Most existing reports are based on European data sources. The objective of this study was to estimate the annual incidence and prevalence of acromegaly in a large US managed care population, overall and stratified by age, sex, and geographic region, using data from 2008 to 2012. Methods Using administrative claims data, commercial health plan enrollees were identified with acromegaly if they had tw...

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

    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...

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

    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.

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

    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.

  7. Pasireotide: a novel treatment for patients with acromegaly

    Cuevas-Ramos D

    2016-01-01

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

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

    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

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

    Idowu Olufemi Emmanuel

    2015-01-01

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

  10. Acromegaly associated with a symptomatic Rathke′s cyst

    Vishal Gupta

    2011-01-01

    Full Text Available Our aim is to describe a case of acromegaly that was associated with symptomatic Rathke′s cyst. We describe a young male student without any significant family history who presented with clinical and biochemical features consistent with growth hormone excess, which was confirmed with dynamic testing. He also described a persistent headache predating symptoms of growth hormone excess by 4 years. Magnetic resonance imaging (MRI of the pituitary showed a large sellar mass which was thought to be a somatotroph adenoma. Trans-sphenoidal surgery was performed; however, a colloid lesion was identified by the neurosurgeon that proved to be a Rathke′s cyst. The association of acromegaly with Rathke′s cyst is very rare, with less than 10 cases found to be reported on review of literature. This is the first report from India.

  11. Locking of metacarpophalangeal joints in a patient with acromegaly

    Tani, Y.; Tanaka, N.; Isoya, Eiji [Dept. of Orthopaedic Surgery, Soseikai General Hospital, Kyoto (Japan)

    1999-11-01

    A 39-year-old man with acromegaly exhibited locking of metacarpophalangeal (MCP) joints of both index fingers. Large osteophytes were found at the metacarpal heads by radiography and computerized tomography (CT). Magnetic resonance (MR) images revealed hypertrophy of volar plates. We suggest that these characteristic acromegalic features caused locking of MCP joints. Surgery was required on one of the joints to release the locking. (orig.)

  12. Acromegaly Quality of Life Questionnaire (AcroQoL

    Lara Nuria

    2004-02-01

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

  13. Correlation between GH and IGF-1 during treatment for acromegaly.

    Oldfield, Edward H; Jane, John A; Thorner, Michael O; Pledger, Carrie L; Sheehan, Jason P; Vance, Mary Lee

    2016-11-18

    OBJECTIVE The relationship between growth hormone (GH) and insulin-like growth factor-1 (IGF-1) in patients with acromegaly as serial levels drop over time after treatment has not been examined previously. Knowledge of this relationship is important to correlate pretreatment levels that best predict response to treatment. To examine the correlation between GH and IGF-1 and IGF-1 z-scores over a wide range of GH levels, the authors examined serial GH and IGF-1 levels at intervals before and after surgery and radiosurgery for acromegaly. METHODS This retrospective analysis correlates 414 pairs of GH and IGF-1 values in 93 patients with acromegaly. RESULTS Absolute IGF-1 levels increase linearly with GH levels only up to a GH of 4 ng/ml, and with IGF-1 z-scores only to a GH level of 1 ng/ml. Between GH levels of 1 and 10 ng/ml, increases in IGF-1 z-scores relative to changes in GH diminish and then plateau at GH concentrations of about 10 ng/ml. From patient to patient there is a wide range of threshold GH levels beyond which IGF-1 increases are no longer linear, GH levels at which the IGF-1 response plateaus, IGF-1 levels at similar GH values after the IGF-1 response plateaus, and of IGF-1 levels at similar GH levels. CONCLUSIONS In acromegaly, although IGF-1 levels represent a combination of the integrated effects of GH secretion and GH action, the tumor produces GH, not IGF-1. Nonlinearity between GH and IGF-1 occurs at GH levels far below those previously recognized. To monitor tumor activity and tumor viability requires measurement of GH levels.

  14. Skin manifestations of acromegaly - a study of 34 cases.

    Arya, K R; Krishna, K; Chadda, M

    1997-01-01

    The common dermatological manifestations seen in 34 cases of acromegaly were changes in facial appearance, enlargement of hands and feet, intolerance to heat and sweating, carpal tunnel syndrome, hirsutism, acrochordons and acanthosis nigricans. The mean estimated age of onset was 32.8 years in males and 31.7 years in females, while the mean age at the time of diagnosis was 38.6 years and 36.1 years for males and females respectively, with a slight male preponderance noted.

  15. Acromegaly with sleep disturbances relieved by yttrium-90 pituitary implantation

    Rosenstock, J.; Doyle, F.H.; Joplin, G.F.; Jung, R.T.; Mashiter, K. (Hammersmith Hospital, London (UK). Postgraduate Medical School)

    1982-03-01

    A brief case history is presented of a patient, who, after yttrium-90 implantation, showed a complete clinical and hormonal remission of her acromegaly, maintaining normal pituitary function. The remarkable feature was the rapid disappearance of her attacks of somnolence within 96 hours of pituitary implantation, despite persistence of nocturnal snoring and well before any remodelling of soft tissues could have occurred. This response suggests that her daytime somnolence had a narcoleptic component.

  16. Effects of previous growth hormone excess and current medical treatment for acromegaly on cognition

    Brummelman, Pauline; Koerts, Janneke; Dullaart, Robin P. F.; van den Berg, Gerrit; Tucha, Oliver; Wolffenbuttel, Bruce H. R.; van Beek, Andre P.

    2012-01-01

    Background In untreated acromegaly patients, decreased cognitive functioning is reported to be associated with the degree of growth hormone (GH) and IGF-1 excess. Whether previous GH excess or current medical treatment for acromegaly specifically affects cognition remains unclear. The aim of this st

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

    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.

  18. Surgical efficacy of carpal tunnel release for carpal tunnel syndrome in acromegaly: report of four patients.

    Iwasaki, N; Masuko, T; Ishikawa, J; Minami, A

    2005-12-01

    Although carpal tunnel syndrome is frequent in acromegaly, few acromegalics will be encountered by most hand surgeons. This paper considers the treatment of four cases of acromegaly in whom carpal tunnel syndrome arose, to discuss aspects of management of carpal tunnel syndrome in this patient group.

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

    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.

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

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

    2016-04-01

    Klinefelter syndrome (KS) is the most common chromosomal aneuploidy in male population, which demonstrates an unusual association with acromegaly. We herein present a rare case involving the confirmation of KS 2 years after surgical treatment for acromegaly.A 27-year-old man presented with an acromegalic appearance. Endocrinological examination revealed a high growth hormone (GH) concentration, low testosterone concentration, and high follicle-stimulating hormone and luteinizing hormone concentration. Brain imaging revealed a 9 × 6 × 7- mm sellar low-density nodule suggestive of a microadenoma. Trans-sphenoidal surgery was undertaken, and immunohistochemistry revealed GH positivity. Two years after surgery, the patient underwent examination for infertility. He presented with diminished pubic hair, and small and firm testes. Hormonal assay revealed hypergonadotrophic hypogonadism on the basis of decreased serum total testosterone (syndrome itself. As the simultaneous occurrence of KS and acromegaly is rare, and the association between them remains unclear, we suggest that complete pituitary hormonal screening and conventional pituitary MRI should be essential for patients with KS to screen for pituitary tumor.

  1. Morphologic study of microcirculation in acromegaly by capillaroscopy.

    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.

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

    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.

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

    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.

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

    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.

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

    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.

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

    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.

  7. [Carpal tunnel syndrome in acromegaly--4-case report and review of literature].

    Imamura, H; Isu, T; Iwasaki, Y; Sugimoto, S; Abe, H; Tashiro, K

    1989-09-01

    Four cases of carpal tunnel syndrome in acromegaly were reported. These 4 cases were found in 21 acromegalies (19%). Besides change of features, they complained bilateral sensory disturbances of their hands. After transsphenoidal removal of pituitary adenoma, GH levels returned to the normal range and sensory disturbances were improved in all cases. Mechanism of carpal tunnel syndrome in acromegaly is that edematous synovial tissues compress the median nerve because oversecretion of growth hormone causes increase of sodium and water retention in the extracellular fluid. The patient who showes high basal level of growth hormone and/or acromegalic pattern by various tolerance tests does not always have the carpal tunnel syndrome. But this syndrome is apt to be found in active acromegaly. So the detection of the symptom showed by this activity such as hypersudation in our cases leads to the early diagnosis of acromegaly.

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

    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

  9. The prevalence of acromegaly in hospitalized patients with type 2 diabetes.

    Suda, Kentaro; Fukuoka, Hidenori; Iguchi, Genzo; Hirota, Yushi; Nishizawa, Hitoshi; Bando, Hironori; Matsumoto, Ryusaku; Takahashi, Michiko; Sakaguchi, Kazuhiko; Takahashi, Yutaka

    2015-01-01

    The prevalence of acromegaly is estimated to be 8-24/100,000, but several recent studies suggest it is underestimated. In particular, acromegaly is considered more prevalent in patients with type 2 diabetes mellitus (T2DM) than in the normal population. This study aimed to evaluate the prevalence of acromegaly in hospitalized patients with T2DM. A total of 327 hospitalized patients with T2DM were recruited as subjects. If serum insulin-like growth factor 1 (IGF-1) levels were found to be elevated, random GH level was measured or oral glucose tolerance test (OGTT) was performed. Five patients with elevated serum IGF-1 levels and random GH level or inadequate suppression of GH in the OGTT underwent pituitary magnetic resonance imaging. Of those patients, pituitary adenoma was detected in 2 patients. These 2 patients were diagnosed with acromegaly, as they also exhibited mild acromegalic features. Intriguingly, both these patients exhibited severe macroangiopathy and an absence of microangiopathy. The prevalence of acromegaly in the hospitalized patients with T2DM in this study was therefore 0.6%, suggesting a higher prevalence than that predicted. Although a large-scale prospective study is required to clarify the precise prevalence of acromegaly in hospitalized patients with T2DM, the present study shows that it is useful to screen hospitalized patients with T2DM for acromegaly by measuring their serum IGF-1 level.

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

    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.

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

    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.

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

    Christofides, Elena A

    2016-01-01

    In acromegaly, achieving biochemical control (growth hormone [GH] level 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

  13. Screening for Acromegaly in Patients with Carpal Tunnel Syndrome: A Prospective Study (ACROCARP).

    Zoicas, F; Kleindienst, A; Mayr, B; Buchfelder, M; Megele, R; Schöfl, C

    2016-07-01

    Early diagnosis of acromegaly prevents irreversible comorbidities and facilitates surgical cure. Carpal tunnel syndrome (CTS) is common in acromegaly and patients have often undergone surgery for CTS prior to the diagnosis of acromegaly. We hypothesized that screening CTS-patients for acromegaly could facilitate active case-finding. We prospectively enrolled 196 patients [135 women, 56.9 (range 23-103) years] who presented with CTS for surgery. Patients were asked about 6 symptoms suggestive of acromegaly using a questionnaire calculating a symptom score (0-6 points), and insulin-like-growth factor 1 (IGF-1) was measured. If IGF-1 was increased, IGF-1 measurement was repeated, and random growth hormone (GH) and/or an oral glucose tolerance test (OGTT) with assessment of GH-suppression were performed. The mean symptom score was 1.7±1.3 points. Three patients reported the maximal symptom score of 6 points, but none of them had an increased IGF-1. There was no correlation between the symptom score and IGF-1-SDS (standard deviation score) (r=0.026; p=0.71). Four patients had an IGF-1>2 SDS. In 2 patients acromegaly was ruled out using random GH and OGTT. One patient had normal IGF-1 and random GH at follow-up. One patient refused further diagnostics. In this prospective cohort of patients with CTS, the observed frequency of acromegaly was at most 0.51% (95% CI 0.03 to 2.83%). In this prospective study, none of the 196 patients with CTS had proven acromegaly. Thus, we see no evidence to justify general screening of patients with CTS for acromegaly.

  14. The Modern Criteria for Medical Management of Acromegaly.

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

    2016-01-01

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

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

    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.

  16. Endoscopic versus microscopic approach for surgical treatment of acromegaly.

    Fathalla, Hussein; Cusimano, Michael D; Di Ieva, Antonio; Lee, John; Alsharif, Omar; Goguen, Jeannette; Zhang, Stanley; Smyth, Harley

    2015-07-01

    Transsphenoidal surgery in the setting of acromegaly is quite challenging due to increased soft tissue mass, bony overgrowth, and bleeding. There is a debate on the endoscopic versus microscopic approach for these patients. The purpose of our study is to compare the outcomes for acromegaly after transsphenoidal surgery using both techniques. Retrospective review of 65 acromegalic patients who underwent transsphenoidal surgery in our department. Clinical remission was defined as resolution of typical acromegalic symptoms. Radiological resection was defined by volumetric criteria, and biochemical remission was defined as by the 2010 consensus on the criteria for remission of acromegaly. There was no significant difference in age, preoperative endocrine status, percent of macro adenomas, suprasellar, or infrasellar extension between both groups. Patients were assigned to both groups based on our existing referral pattern. Endoscopic approach was performed in 42 patients, while the microscopic approach was performed in 23 patients. No significant difference in remission rates was found between both groups (45.2 vs. 34.7 %, p = 0.40). The endoscopic group, however, had a significantly higher rate of gross total resections (61 vs. 42 %, p = 0.05). There was also a trend towards higher rates of gross total resections when cavernous sinus was present (48 vs. 14.2 %, p = 0.09). Postoperative diabetes insipidus occurred more in microscopic patients (34.7 vs. 17 %, p = 0.05), otherwise there was no significant difference in rates of complications. The median follow-up period was 56.6 months (range 6-156, mean 66.1). There is no significant difference in the rates of biochemical remission between the endoscopic and microscopic techniques. The endoscope technique, however, seems to be superior in achieving gross total resection especially with tumors invading the cavernous sinus.

  17. Skin manifestations of acromegaly - a study of 34 cases

    Arya Kavita

    1997-01-01

    Full Text Available The common dermatological manifestations seen in 34 cases of acromegaly were changes in facial appearance, enlargement of hands and feet, intolerance to heat and sweating, carpal tunnel syndrome, hirsutism, acrochordons and acanthosis nigricans. The mean estimated age of onset was 32.8 years in males and 31.7 years in females, while the mean age at the time of diagnosis was 38.6 years and 36.1 years for males and females respectively, with a slight male preponderance noted.

  18. The Oldest Recorded Case of Acromegaly and Gigantism in Iran.

    Najjari, Mohsen

    2015-10-01

    Here we commemorate the character and academic authority of Prof. Zabiholah Gorban (1903-2006), the founder of Shiraz medical school. No doubt, in the scope of history of contemporary medicine, he has been efficient and effective. With respect to this fact, his article on a rare case described in Acta anatomica published in Iran in 1966, entitled (Observations on a giant skeleton) is browsed and reviewed. A case named Siah Khan with combined acromegaly and gigantism that appears to have letters to say still after nearly half a century.

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

    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.

  20. Risk factors for glucose intolerance in active acromegaly

    Kreze A.

    2001-01-01

    Full Text Available In the present retrospective study we determined the frequency of glucose intolerance in active untreated acromegaly, and searched for risk factors possibly supporting the emergence of the diabetic condition. Among 43 patients, 8 (19%; 95% CI: 8-33% had diabetes mellitus and 2 (5%; 1-16% impaired glucose tolerance. No impaired fasting glycemia was demonstrable. The frequency of diabetes was on average 4.5 times higher than in the general Slovak population. Ten factors suspected to support progression to glucose intolerance were studied by comparing the frequency of glucose intolerance between patients with present and absent risk factors. A family history of diabetes and arterial hypertension proved to have a significant promoting effect (P<0.05, chi-square test. A significant association with female gender was demonstrated only after pooling our data with literature data. Concomitant prolactin hypersecretion had a nonsignificant promoting effect. In conclusion, the association of active untreated acromegaly with each of the three categories of glucose intolerance (including impaired fasting glycemia, not yet studied in this connection was defined as a confidence interval, thus permitting a sound comparison with the findings of future studies. Besides a family history of diabetes, female gender and arterial hypertension were defined as additional, not yet described risk factors.

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

    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.

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

    Yusuf Aydın

    2009-06-01

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

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

    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.

  4. AcroBel - the Belgian registry on acromegaly : a survey of the 'real-life' outcome in 418 acromegalic subjects

    Bex, Marie; Abs, Roger; T'Sjoen, Guy; Mockel, Lean; Velkeniers, Brigitte; Muermans, Katja; Maiter, Dominique

    2007-01-01

    Objectives: To constitute a registry on acromegaly, AcroBel, to evaluate the epidemiology and quality of care of acromegaly in Belgium and Luxembourg. Design: A nationwide survey from June 2003 till September 2004 aiming to collect data from all patients with acromegaly who had visited the participating endocrine clinics after 1 January 2000. Methods: Retrospective data collection coupled to a visit within the survey period, allowing sampling of metabolic parameters and centralised dete...

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

    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...

  6. Intraoperation haemorrhage into hypophysis adenoma as the cause of acromegaly remission

    V N Azizyan

    2011-03-01

    Full Text Available In this article we describe a spontaneous remission of acromegaly of intraoperative bleeding, with subsequent hemorrhage into the tumor. The cases of spontaneous remission of acromegaly described in the literature have been associated mainly with hemorrhage or ischemic apoplexy pituitary adenoma without surgical intervention. Most often, both processes, especially hemorrhage are accompanied by the development of panhypopituitarism. Cases in which there was a normalization of only growth hormone isolated.

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

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

    2007-01-01

    vs. 1563 +/- 101 kcal/24 h, P = 0.03), but the rate of lipid oxidation did not change significantly. CONCLUSIONS: 1) Pegvisomant treatment for 4 wk improves peripheral and hepatic insulin sensitivity in acromegaly. 2) This is associated with a decrease in resting energy expenditure, whereas free...... fatty acid metabolism is unaltered. 3) The data support the important direct effects of GH on glucose metabolism and add additional benefits to pegvisomant treatment for acromegaly...

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

    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...

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

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

    2012-01-01

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

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

    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...

  11. Rosiglitazone as an option for patients with acromegaly: a case series

    Bahena-García Ana; Tamez-Pérez Héctor E; Gómez de Ossio María D; Gutiérrez-Hermosillo Hugo; Tamez-Peña Alejandra L

    2011-01-01

    Abstract Introduction In the patient with acromegaly, pituitary surgery is the therapeutic standard. Despite undergoing surgery, a significant number of patients with acromegaly continue to have uncontrolled growth hormone secretion. These patients require other treatments such as external irradiation and/or drug therapy. Case presentation We present the clinical and laboratory responses to six months of treatment with rosiglitazone in four cases. In all four cases, the patients had persisten...

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

    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.

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

    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.

  14. Increased Prevalence of Colorectal Polyp in Acromegaly Patients: A Case-Control Study

    Ali Riza Koksal

    2014-01-01

    Full Text Available An increase in the prevalence of colorectal polyps and cancer is reported in patients with acromegaly. This trial is designed to determine whether there is an increase in the prevalence of colorectal polyps/cancer in Turkish acromegaly patients. Sixty-six patients, who were under follow-up with the diagnosis of acromegaly and underwent total colonoscopic examination, were enrolled in the study. Sixty-five age- and gender-matched patients with nonspecific complaints were selected as control. The mean age of acromegalic patients was 51.5±12.8 years of whom 27 (40.9% were females. In 20 (30.3% of the patients with acromegaly a total of 65 colorectal polyps were detected. Forty-seven (72.3% of the polyps were detected at the rectosigmoid region. In 8 (12.3% of the 65 control patients a total of 17 polyps were found. There was a statistically significant difference between the groups (P=0.018. At the logistic regression analysis we found that the risk for colon polyps increased 3.2-fold in the presence of acromegaly, irrespective of age and gender (OR: 3.191, 95% CI: 1.25–8.13. In conclusion, patients who were followed up with the diagnosis of acromegaly should be taken to the colonoscopic surveillance program and all polyps detected should be excised in order to protect them from colorectal cancer.

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

    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.

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

    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. The Role of Diabetes in Acromegaly Associated Neoplasia

    Cheng, Sonia; Gomez, Karen; Serri, Omar; Chik, Constance; Ezzat, Shereen

    2015-01-01

    Background The risk and mortality due to cancer in patients with acromegaly have been previously investigated. Although GH/IGF-1 excess provides a probable pathophysiological explanation, the degree of IGF-1 excess and the role in acromegaly-associated neoplasms of diabetes, a common comorbidity in acromegaly with known association with cancer, remains unclear. Methods Acromegalic patients treated in three Canadian referral centers (Toronto, Montreal, Edmonton) were included. All available clinical information was recorded including: age, initial and last percentage of the upper limit of normal (%ULN) IGF-1 levels, comorbidities and other neoplasms (benign and malignant). Results 408 cases were assessed. 185 were women (45.3%), 126 (30.9%) developed extra-pituitary neoplasms: 55 malignant and 71 benign. The most frequent anatomic site was the gastrointestinal tract (46 [11.3%]), followed by head and neck (36 [8.8%]) and multiple locations (14 [3.4%]). 106 (26.0%) cases had diabetes. Initial IGF-1 was significantly higher in men older than 50 (380.15 vs. 284.78, p = 0.001) when compared to men younger than 50. Diabetics showed significantly higher initial IGF-1 (389.38 vs. 285.27, p = 0.009), as did diabetics older than 50 compared with those without diabetes. 45.3% (48/106) of cases with diabetes developed extra-pituitary neoplasms vs. 24.3% (71/292) without diabetes (p = 0.001, OR: 2.576 95%CI 1.615–4.108). 22.6% (24/106) of cases with diabetes developed malignant tumors vs. 9.2% (27/292), (p < 0.001, OR 2.873, 95%CI 1.572–5.250). Conclusions These data suggest that acromegalic patients with diabetes are more likely to develop extra-pituitary neoplasms and their initial IGF-1 levels are higher. The contribution of IGF-1 vs. diabetes alone or in combination in the development of extra-pituitary neoplasms warrants further investigation. PMID:25996963

  18. The role of diabetes in acromegaly associated neoplasia.

    Sonia Cheng

    Full Text Available The risk and mortality due to cancer in patients with acromegaly have been previously investigated. Although GH/IGF-1 excess provides a probable pathophysiological explanation, the degree of IGF-1 excess and the role in acromegaly-associated neoplasms of diabetes, a common comorbidity in acromegaly with known association with cancer, remains unclear.Acromegalic patients treated in three Canadian referral centers (Toronto, Montreal, Edmonton were included. All available clinical information was recorded including: age, initial and last percentage of the upper limit of normal (%ULN IGF-1 levels, comorbidities and other neoplasms (benign and malignant.408 cases were assessed. 185 were women (45.3%, 126 (30.9% developed extra-pituitary neoplasms: 55 malignant and 71 benign. The most frequent anatomic site was the gastrointestinal tract (46 [11.3%], followed by head and neck (36 [8.8%] and multiple locations (14 [3.4%]. 106 (26.0% cases had diabetes. Initial IGF-1 was significantly higher in men older than 50 (380.15 vs. 284.78, p = 0.001 when compared to men younger than 50. Diabetics showed significantly higher initial IGF-1 (389.38 vs. 285.27, p = 0.009, as did diabetics older than 50 compared with those without diabetes. 45.3% (48/106 of cases with diabetes developed extra-pituitary neoplasms vs. 24.3% (71/292 without diabetes (p = 0.001, OR: 2.576 95%CI 1.615-4.108. 22.6% (24/106 of cases with diabetes developed malignant tumors vs. 9.2% (27/292, (p < 0.001, OR 2.873, 95%CI 1.572-5.250.These data suggest that acromegalic patients with diabetes are more likely to develop extra-pituitary neoplasms and their initial IGF-1 levels are higher. The contribution of IGF-1 vs. diabetes alone or in combination in the development of extra-pituitary neoplasms warrants further investigation.

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

    Hee Kyung Kim

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

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

    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

  1. Systolic and diastolic cardiac function in acromegaly. An echocardiographic study.

    Galanti, G; Cappelli, B; Diricatti, G; Mininni, S; Vono, M C; Gensini, G F

    1996-01-01

    The aim of this study was to establish the existence of primary acromegalic cardiomyopathy different from the cardiovascular complications often associated with acromegaly. Thirty-four acromegalic patients, referred to our non-invasive laboratory and divided into two groups on the basis of the presence of hypertension, underwent echocardiographic studies. A control group of 34 subjects individually matched with the patients for age, sex, and blood pressure values was also studied. To evaluate cardiac function during exercise, the normotensive acromegalics, the control group, and a group of 9 athletes with left ventricular mass comparable to that of the acromegalic subjects underwent a handgrip test. Cardiac mass was increased in all patients; hypertensive patients had a greater increase than normotensive patients (144.9 +/- 38 vs 120.9 +/- 20.8 g/m, p cardiac hypertrophy caused by GH hyperincretion does not improve acromegalic heart activity: diastolic function, although normal at rest, appears deficient during isometric exercise.

  2. Ruptured chordae tendineae in acromegaly. An autopsy case.

    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.

  3. Surgical orthodontic correction of acromegaly with mandibular prognathism.

    Yagi, Takakaza; Kawakami, Masayoshi; Takada, Kenji

    2004-02-01

    A male (30 years five months) who complained of mandibular prominence and masticatory dysfunction was diagnosed as a mandibular prognathic with acromegaly after cephalometric and endocrine examinations. The level of growth hormone (GH) subsequent to a transsphenoidal hypophysectomy had been controlled by medicines for about five years. Surgical orthodontic correction improved his occlusion and profile, but magnetic resonance imaging detected a recurrent adenoma in the cranial base during the retention period. The recurrence resulted in slight prognathic changes of the patient with a high level of GH. This is a case report of the treatment of an acromegalic patient discussing growth considerations that could influence the orthodontic treatment plan and long-term stability.

  4. Dissociated hypopituitarism after spontaneous pituitary apoplexy in acromegaly

    Shahnaz Ahmad Mir

    2013-01-01

    Full Text Available Introduction: Pituitary apoplexy is an uncommon event and usually occurs in non-functioning pituitary tumors. Among the functioning tumors, prolactinomas are the ones most likely to apoplexy. Apoplexy in growth hormone (GH producing adenomas is a very rare event with less than thirty cases reported worldwide. Objective: To describe a case of spontaneous pituitary apoplexy in acromegaly. Case Report: A 55 year old smoker male presented to the our outpatient clinic in 2004 with complaints of gradual onset increase in the size of hands and feet, bilateral knee pain, increased sweating and blurring of vision. Investigations uncovered diabetes mellitus by a casual blood glucose of 243 mg/dl and HbA1c of 8.5%. Growth hormone suppression test using 75 gram oral glucose showed a 60 minute growth hormone of 105 ng/ml. Magnetic resonance imaging of the sellar region showed a 12.0 mm × 10.0 mm pituitary adenoma. The patient was planned for transsphenoidal tumor decompression. However, the patient was lost to follow up. Eight-years later, he presented in the emergency department of our institute with sudden onset headache, vomiting and decreased level of consciousness of one day duration. CT scan of the head with focus on the sella was suggestive of apoplexy which was later confirmed by the MRI of the sellar region. Conclusion: Although acromegaly can remit following apoplexy of the responsible pituitary adenoma, long term follow up is needed for early detection of the development of deficiency of pituitary hormones which may occur over years following the event as well as to detect tumor regrowth which again may occur several years later.

  5. Vitamin D-binding protein and free vitamin D concentrations in acromegaly.

    Altinova, Alev Eroglu; Ozkan, Cigdem; Akturk, Mujde; Gulbahar, Ozlem; Yalcin, Muhittin; Cakir, Nuri; Toruner, Fusun Balos

    2016-05-01

    Free 25-hydroxyvitamin D [25(OH)D] is suggested to be important in the determination of vitamin D deficiency, since vitamin D-binding protein (VDBP) may affect total 25(OH)D levels. There are no data about free 25(OH)D concentrations in acromegaly. We aimed to investigate serum VDBP and total and free 25(OH)D levels in patients with acromegaly in comparison with control subjects. We recruited 54 patients with acromegaly and 32 control subjects who were similar according to age, gender, and body mass index. Serum VDBP levels were found to be increased in patients with acromegaly compared to control subjects [90.35 (72.45-111.10) vs. 69.52 (63.89-80.13) mg/l, p = 0.001]. There was statistically no significant difference in serum total 25(OH)D levels between the patients with acromegaly and control subjects [18.63 (13.35-27.73) vs. 22.51 (19.20-28.96) ng/ml, p = 0.05]. Free 25(OH)D levels were significantly decreased in patients with acromegaly compared to control subjects [14.55 (10.45-21.45) vs. 17.75 (15.30-23.75) pg/ml, p = 0.03]. Free 25(OH)D levels correlated positively with total 25(OH)D (p = 0.0001) and HDL cholesterol (p = 0.04) and negatively with fasting blood glucose (p = 0.04). Our findings indicate that VDBP is increased and free 25(OH)D is decreased in acromegaly, while there is no significant alteration in total 25(OH)D.

  6. Adipose Tissue Redistribution and Ectopic Lipid Deposition in Active Acromegaly and Effects of Surgical Treatment

    Reyes-Vidal, Carlos M.; Mojahed, Hamed; Shen, Wei; Jin, Zhezhen; Arias-Mendoza, Fernando; Fernandez, Jean Carlos; Gallagher, Dympna; Bruce, Jeffrey N.; Post, Kalmon D.

    2015-01-01

    Context: GH and IGF-I have important roles in the maintenance of substrate metabolism and body composition. However, when in excess in acromegaly, the lipolytic and insulin antagonistic effects of GH may alter adipose tissue (AT) deposition. Objectives: The purpose of this study was to examine the effect of surgery for acromegaly on AT distribution and ectopic lipid deposition in liver and muscle. Design: This was a prospective study before and up to 2 years after pituitary surgery. Setting: The setting was an academic pituitary center. Patients: Participants were 23 patients with newly diagnosed, untreated acromegaly. Main Outcome Measures: We determined visceral (VAT), subcutaneous (SAT), and intermuscular adipose tissue (IMAT), and skeletal muscle compartments by total-body magnetic resonance imaging, intrahepatic and intramyocellular lipid by proton magnetic resonance spectroscopy, and serum endocrine, metabolic, and cardiovascular risk markers. Results: VAT and SAT masses were lower than predicted in active acromegaly, but increased after surgery in male and female subjects along with lowering of GH, IGF-I, and insulin resistance. VAT and SAT increased to a greater extent in men than in women. Skeletal muscle mass decreased in men. IMAT was higher in active acromegaly and decreased in women after surgery. Intrahepatic lipid increased, but intramyocellular lipid did not change after surgery. Conclusions: Acromegaly may present a unique type of lipodystrophy characterized by reduced storage of AT in central depots and a shift of excess lipid to IMAT. After surgery, this pattern partially reverses, but differentially in men and women. These findings have implications for understanding the role of GH in body composition and metabolic risk in acromegaly and other clinical settings of GH use. PMID:26037515

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

    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

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

    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

  9. Acromegaly caused by a growth hormonereleasing hormone secreting carcinoid tumour of the lung : the effect of octreotide treatment

    De Heide, L. J. M.; Van den Berg, G.; Wolthuis, A.; Van Schelven, W. D.

    2007-01-01

    in acromegaly, the overproduction of growth hormone is usually caused by a pituitary adenoma. We report a 74-year-old woman with acromegaly caused by ectopic overproduction of growth hormone-releasing hormone (GHRH), a rare diagnosis. The GHRH appeared to be produced by a carcinoid tumour of the lun

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

    Sherlock, M

    2009-11-01

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

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

    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.

  12. Acromegaly with Normal Insulin-Like Growth Factor-1 Levels and Congestive Heart Failure as the First Clinical Manifestation.

    Lee, Hyae Min; Lee, Sun Hee; Yang, In Ho; Hwang, In Kyoung; Hwang, You Cheol; Ahn, Kyu Jeung; Chung, Ho Yeon; Hwang, Hui Jeong; Jeong, In Kyung

    2015-09-01

    The leading cause of morbidity and mortality in patients with acromegaly is cardiovascular complications. Myocardial exposure to excessive growth hormone can cause ventricular hypertrophy, hypertension, arrhythmia, and diastolic dysfunction. However, congestive heart failure as a result of systolic dysfunction is observed only rarely in patients with acromegaly. Most cases of acromegaly exhibit high levels of serum insulin-like growth factor-1 (IGF-1). Acromegaly with normal IGF-1 levels is rare and difficult to diagnose. Here, we report a rare case of an acromegalic patient whose first clinical manifestation was severe congestive heart failure, despite normal IGF-1 levels. We diagnosed acromegaly using a glucose-loading growth hormone suppression test. Cardiac function and myocardial hypertrophy improved 6 months after transsphenoidal resection of a pituitary adenoma.

  13. Acromegaly with Normal Insulin-Like Growth Factor-1 Levels and Congestive Heart Failure as the First Clinical Manifestation

    Hyae Min Lee

    2015-09-01

    Full Text Available The leading cause of morbidity and mortality in patients with acromegaly is cardiovascular complications. Myocardial exposure to excessive growth hormone can cause ventricular hypertrophy, hypertension, arrhythmia, and diastolic dysfunction. However, congestive heart failure as a result of systolic dysfunction is observed only rarely in patients with acromegaly. Most cases of acromegaly exhibit high levels of serum insulin-like growth factor-1 (IGF-1. Acromegaly with normal IGF-1 levels is rare and difficult to diagnose. Here, we report a rare case of an acromegalic patient whose first clinical manifestation was severe congestive heart failure, despite normal IGF-1 levels. We diagnosed acromegaly using a glucose-loading growth hormone suppression test. Cardiac function and myocardial hypertrophy improved 6 months after transsphenoidal resection of a pituitary adenoma.

  14. Increased activity of digoxin-like substance in low-renin hypertension in acromegaly

    Soszynski, P.; Slowinska-Srzednicka, J.; Zgliczynski, S. (Medical Center for Postgraduate Education, Warsaw (Poland))

    1990-01-01

    Arterial hypertension is common in acromegaly, but the pathogenesis of this complication remains unknown. To determine the role of an endogenous Na,K pump inhibitor/digoxin-like substance (DLS) in the pathogenesis of hypertension in acromegaly 76 subjects: 28 with acromegaly, 20 with essential hypertension and 28 healthy controls were studied. Serum DLS was measured with the use of radioimmunoassay and bioassay by the inhibition of digoxin-sensitive erythrocyte 86-Rb uptake. In acromegaly, the activity of DLS was significantly increased and plasma renin activity decreased in the hypertensive group, as compared with that of the normotensive group and controls. Moreover, DLS was elevated in the low-renin group of essential hypertension, as compared with that of the normal/high-renin group or controls. The activity of DLS correlated positively with mean arterial pressure and negatively with plasma renin activity, but not with growth hormone levels. In conclusion, an endogenous sodium pump inhibitor/digoxin-like substance may play a role in the pathogenesis of low-renin hypertension in acromegaly.

  15. Serum ghrelin levels in acromegaly: effects of surgical and long-acting octreotide therapy.

    Freda, Pamela U; Reyes, Carlos M; Conwell, Irene M; Sundeen, Robert E; Wardlaw, Sharon L

    2003-05-01

    The orexigenic peptide, ghrelin, is regulated by acute and chronic nutritional state. Although exogenously administered ghrelin stimulates pituitary GH secretion, little is known about the role of ghrelin in endogenous GH secretion or how high GH and IGF-I levels in acromegaly could affect ghrelin secretion and vice versa. Therefore, we evaluated fasting and post oral glucose tolerance test serum ghrelin levels in 19 patients with active acromegaly at baseline and after either surgery in 9 of these or administration of long-acting octreotide (Sandostatin LAR) in the other 10 patients. After surgical cure, fasting ghrelin rose from 312 +/- 56 pg/ml to 548 +/- 97 pg/ml (P = 0.013). Fasting serum ghrelin levels were higher in all patients after surgery and ranged between 112% and 349% of presurgery levels. Ghrelin levels fell significantly during long-acting octreotide therapy from 447 +/- 34 pg/ml to 206 +/- 15 pg/ml (P acromegaly; lowered serum levels of ghrelin in active acromegaly rise along with the postsurgery normalization of GH and IGF-I and improved insulin resistance. In contrast to surgical therapy, long-acting octreotide therapy persistently suppressed serum ghrelin levels. It remains to be determined whether altered circulating ghrelin concentrations could impact on body composition changes in acromegaly.

  16. Zinc, copper, manganese, and selenium metabolism in patients with human growth hormone deficiency or acromegaly.

    Aihara, K; Nishi, Y; Hatano, S; Kihara, M; Ohta, M; Sakoda, K; Uozumi, T; Usui, T

    1985-08-01

    This study was designed to evaluate trace metal metabolism in patients with known abnormalities of human growth hormone (hGH). The mean concentration of zinc in plasma and urine decreased in patients with hGH deficiency after hGH injection, whereas, after adenomectomy, in patients with acromegaly, zinc increased in plasma, remained the same in erythrocytes, and decreased in urine. There was a negative correlation between plasma zinc and serum hGH levels and a positive correlation between urinary zinc excretion and serum hGH levels in acromegaly. In hGH deficiency, the copper content remained unchanged in plasma and erythrocytes and rose in urine after treatment; however, in acromegaly, the copper content increased in plasma and remained unchanged in erythrocytes and urine after surgery. The mean concentration of erythrocyte manganese did not change significantly after treatment in patients with hGH deficiency or acromegaly, but the pre-hGH treatment level of erythrocyte manganese in hGH deficiency was lower than in the controls. Plasma selenium concentrations were decreased in hGH deficiency and increased in acromegaly patients after therapy. These results suggest that hGH affects the metabolism of zinc, copper, manganese, and selenium.

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

    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.

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

    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.

  19. Body Image Disturbance in Acromegaly Patients Compared to Nonfunctioning Pituitary Adenoma Patients and Controls

    Helen M. Conaglen

    2015-01-01

    Full Text Available Purpose. Excess growth hormone secretion in adults results in acromegaly, a condition in which multiple physical changes occur including bony and soft tissue overgrowth. Over time these changes can markedly alter a person’s appearance. The aim of this study was to compare body image disturbance in patients with acromegaly to those with nonfunctioning pituitary adenomas (NFAs and controls and assess the impact of obesity in these groups. Methods. A cross-sectional survey including quality of life, body image disturbance, anxiety and depression measures, growth hormone, and BMI measurement was carried out. Results. The groups did not differ with respect to body image disturbance. However separate analysis of obese participants demonstrated relationships between mood scales, body image disturbance, and pain issues, particularly for acromegaly patients. Conclusions. While the primary hypothesis that acromegaly might be associated with body image disturbance was not borne out, we have shown that obesity together with acromegaly and NFA can be associated with body image issues, suggesting that BMI rather than primary diagnosis might better indicate whether patients might experience body image disturbance problems.

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

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

    2002-01-01

    of these growth factors in relation to biomechanical properties in acromegaly. MATERIALS AND METHODS: Trabecular bone biomechanical competence (compression test), apparent density (peripheral quantitative computed tomography, pQCT), and bone matrix contents of calcium (HCl hydrolysis) and IGFs (guanidinium......-HCl extraction) were measured in iliac crest biopsies from 13 patients with active acromegaly (two women and 11 men, aged 21-61 years) and 21 age- and sex-matched controls (four women and 17 men, aged 23-64 years). RESULTS: Trabecular bone pQCT was reduced in acromegalic patients compared with controls (P = 0...... bone content of IGF-I, IGFBP-3, or osteocalcin. However, IGF-II and IGFBP-5 content was decreased (P acromegaly, supporting previous observations...

  1. Acromegaly with no pituitary adenoma and no evidence of ectopic source

    Deepak Khandelwal

    2011-01-01

    Full Text Available More than 99% of patients with acromegaly harbor a growth hormone (GH secreting pituitary adenoma. As the time from onset of signs/symptoms to diagnosis of acromegaly is long (symptom onset to diagnosis is often 4-10 years, pituitary adenomas that cause GH excess are often large and are nearly always visible on conventional magnetic resonance imaging (MRI. However, in rare circumstances, acromegalic patients without an ectopic source will not have imaging evidence of a pituitary adenoma. Management of these patients poses special challenge, and once ectopic source of GH/growth-hormone-releasing hormone (GHRH is ruled out, an exploration of pituitary might be useful. We herein report a case of acromegaly with imaging evidence of sellar floor osteoma, but no pituitary adenoma, and negative work up for an ectopic source of GH/GHRH tumor, and on surgical exploration pituitary adenoma could be identified and removed and confirmed on histopathologic examination.

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

    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.

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

    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.

  4. Rosiglitazone as an option for patients with acromegaly: a case series

    Bahena-García Ana

    2011-05-01

    Full Text Available Abstract Introduction In the patient with acromegaly, pituitary surgery is the therapeutic standard. Despite undergoing surgery, a significant number of patients with acromegaly continue to have uncontrolled growth hormone secretion. These patients require other treatments such as external irradiation and/or drug therapy. Case presentation We present the clinical and laboratory responses to six months of treatment with rosiglitazone in four cases. In all four cases, the patients had persistent growth hormone overproduction despite previous surgical treatment and other conventional therapy. Case 1 is a 57-year-old Caucasian woman, case 2 is a 51-year-old Hispanic man, case 3 is a 32-year-old Hispanic woman, and case 4 is a 36-year-old Hispanic man. In three of these patients, basal and nadir growth hormone and insulin-like growth factor 1 levels were significantly decreased (P P Conclusion Rosiglitazone could be a treatment option in select patients with acromegaly.

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

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

    2016-08-01

    Although aryl hydrocarbon receptor-interacting protein (AIP) mutations are rare in sporadic acromegaly, their prevalence among young patients is nonnegligible. The objectives of this study were to evaluate the frequency of AIP mutations in a cohort of Mexican patients with acromegaly with disease onset before the age of 30 and to search for molecular abnormalities in the AIP gene in teeth obtained from the "Tampico Giant". Peripheral blood DNA from 71 patients with acromegaly (51 females) with disease onset 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.

  6. Long-term outcome of patients with acromegaly and congestive heart failure.

    Bihan, Hélène; Espinosa, Consuelo; Valdes-Socin, Hernan; Salenave, Sylvie; Young, Jacques; Levasseur, Suzanne; Assayag, Patrick; Beckers, Albert; Chanson, Philippe

    2004-11-01

    Cardiovascular complications are a major cause of morbidity and mortality in patients with acromegaly. Normalization of GH secretion is associated with an improvement in structural and functional cardiac abnormalities. However, the long-term cardiac effects of treatment for acromegaly have not been studied in patients who have already developed chronic congestive heart failure (CHF). We reviewed the charts of 330 consecutive patients with acromegaly treated in two French and Belgian centers since 1985. Ten patients with both acromegaly and CHF (eight men, two women, mean age 49.7 yr) were studied retrospectively. One of them was excluded because CHF was due to severe aortic stenosis.CHF (New York Heart Association stages III-IV and echocardiography showing dilated hypokinetic cardiomyopathy with left ventricular systolic dysfunction and a left ventricular ejection fraction less than 45%) was diagnosed before, concomitantly, or after acromegaly in, respectively, two, five, and two patients. Three patients were referred with terminal heart failure requiring transplantation.One patient had transient CHF associated with a hypertensive crisis. The other eight patients had symptomatic chronic CHF. Control of GH hypersecretion failed, totally or partially, in three patients: one had a long-term survival, and the two others died at 1 and 5 yr. Good GH control was achieved in five patients: four of these are still alive 2-16 yr after diagnosis of CHF, their clinical status is stable or improved, and their quality of life is good. Overall, the 1- and 5-yr mortality (or transplantation) rates for patients with chronic symptomatic CHF were 25% (2 of 8 patients) and 37.5% (3 of 8 patients), respectively. In conclusion, less than 3% of acromegalic patients developed CHF in this study. Although effective treatment of acromegaly improved short-term cardiovascular status, its impact on long-term survival is questionable.

  7. Circulating levels of pegvisomant and endogenous growth hormone during prolonged pegvisomant therapy in patients with acromegaly

    Madsen, Michael; Fisker, Sanne; Feldt-Rasmussen, Ulla;

    2014-01-01

    OBJECTIVE: To investigate whether pegvisomant treatment in acromegaly induces gradual elevations in endogenous serum growth hormone (GH) levels and whether serum pegvisomant levels predict the therapeutic outcome. PATIENTS AND METHODS: Seventeen patients (6 women), mean age 46·3 years (range: 23...... correlated with baseline growth hormone levels, whereas no associations between serum pegvisomant and either dose, gender, age or body weight were found. CONCLUSIONS: (1) Serum GH levels increased initially, but remained stable during prolonged pegvisomant treatment in patients with acromegaly, (2) serum...

  8. [Orotracheal intubation of patients with acromegaly using the AirTraq laryngoscope].

    Castañeda Pascual, M; Navarro García, C; Batllori Gastón, M; Anadón Senac, María P; Arrondo Nicolás, J; Martín Vizcaíno, M P

    2011-01-01

    An excess of growth hormone is responsible for the phenotypical characteristics of acromegaly. Tissue hypertrophy and growth also affect the airway, potentially making perioperative management difficult. If tests to foresee the likelihood of difficult airway have limitations affecting their sensitivity, specificity and predictive value even in the normal population, their reliability in patients with acromegaly is still more doubtful. At this time, videoassisted or optical laryngoscopes can offer a way to facilitate intubation in these patients. We report 3 cases in which the AirTraq optical laryngoscope was used to gain a full view of the vocal cords in acromegalic patients scheduled for pituitary surgery by the transsphenoidal route.

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

    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.

  10. Pulmonary epidermoid carcinoma in a patient with acromegaly: a rare entity

    El Aziz, Siham; Chadli, Asma; Obbiba, Atika; El Ghomari, Hassan; Farouqi, Ahmed

    2012-01-01

    A 56-years-old woman was referred to our unit for partially treated acromegaly. She had a high level of insulin growth factor. She did not complain of any pulmonary symptoms and was a non-smoker. Physical examination revealed clinical features of acromegaly. She had a 13 mm pituitary adenoma and was proposed for surgical intervention. Her chest X-ray showed a right paracardiac tumor. Computed tomography scan revealed a large right-sided fowler tumor. Pituitary surgery was cancelled and lobect...

  11. [CLINICAL, BIOCHEMICAL AND HORMONAL PREDICTORS OF LEFT VENTRICULAR HYPERTROPHY IN PATIENTS WITH ACROMEGALY].

    Mykytyuk, M R

    2015-01-01

    Examined 76 (26 men and 50 women) patients with active acromegaly aged 20 to 70 years, average age (48.22 ± 12.19). Echocardiographic signs of hypertrophy of the left ventricular (LVH) revealed by 63.2% of patients, including 46%--concentric LVH. Found that high levels of pituitary growth hormone (GH) and insulin like growth factor-1 (IGF-1) were independent predictors of LVH. Influence of GH and IGF-1 on the formation of LVH-mediated through anthropometric parameters and levels of systolic and diastolic blood pressure, which are predictors in patients with acromegaly LVH.

  12. The neuromuscular features of acromegaly: a clinical and pathological study.

    Khaleeli, A A; Levy, R D; Edwards, R H; McPhail, G; Mills, K R; Round, J M; Betteridge, D J

    1984-09-01

    A study of the neuromuscular features of acromegaly was performed in six patients. Clinical assessment was supplemented by quadriceps force measurements, plasma creatine kinase (CK) activities, electromyography (EMG) and nerve conduction studies. Muscle mass was measured by urinary creatinine/height indices (CHI) and cross sectional area (CSA) of thighs and calves on computed tomography. Quadriceps force/unit cross sectional area was derived. Needle biopsies of vastus lateralis were studied by histochemical and ultrastructural methods. Mean fibre area (MFA) and fibre type proportions were measured. Most of the subjects studied had muscle pain and proximal muscle weakness confirmed by quadriceps force measurements. This occurred in the absence of muscle wasting, as shown by cross sectional area measurements and normal or raised creatinine/height indices. "Myopathic" features were demonstrated by needle biopsy in half the patients and occasionally by electromyography and raised plasma creatine kinase activity. Abnormalities on needle biopsy included variation in fibre size, type 2 fibre atrophy and large type 1 MFA relative to type 2 MFA. Electronmicroscopy showed the non-specific findings of increased glycogen accumulation, excess lipofuscin pigment and myofilament loss.

  13. [Multihormonal and multifunctional hypophyseal adenoma and the acromegaly syndrome].

    Dusková, J; Marek, J; Povýsil, C

    2000-07-19

    Woman 75-year-old treated 30 years for syndrome of acromegaly refused pituitary surgery and irradiation. Five years and nine months before death she had a colon carcinoma successfully removed. Multinodular hyperfunctional goitre was treated with carbimazole. For six last years of life corticosteroids were given as a replacement therapy. Her cause of death was the heart failure due to acromegalic heart disease. In autopsy a large intrasellar and extrasellar pituitary adenoma without rests of nonneoplastic tissue was found. Nevertheless the target peripheral endocrine glands except ovaries, were not atrophic. A multinodular goitre and diffuse adrenocortical hyperplasia were revealed. Histology, and immunohistochemistry demonstrated that mot neoplastic cells were producing GH and ACTH, dispersly Prl, scattered cells were positive for beta-subunit of FSH, LH, TSH. Electron microscopy proved most of the cells to be densely granulated. We classify the adenoma according to the newly proposed WHO pituitary tumours classification (1) as plurihormonal, hyperfunctional, extrasellar, typical adenoma from densely granulated cells. We conclude that in plurihormonal adenomas with dominant (in the case referred acromegalic) symptomatology the additional hormonal production should be monitored as a possible source of important complications.

  14. Active postoperative acromegaly: sustained remission after discontinuation of somatostatin analogues

    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.

  15. Acromegaly: Role of Surgery in the Therapeutic Armamentarium

    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.

  16. Assessment of the awareness and management of sleep apnea syndrome in acromegaly. The COM.E.TA (Comorbidities Evaluation and Treatment in Acromegaly) Italian Study Group.

    De Menis, E; Giustina, A; Colao, A; Degli Uberti, E; Ghigo, E; Minuto, F; Bogazzi, F; Drigo, R; Cattaneo, A; Aimaretti, G

    2011-01-01

    In 2007 the Italian COM.E.T.A. (COMorbidities Evaluation and Treatment in Acromegaly) study group started to assess the application in a clinical setting of the Versailles criteria for management of acromegaly complications by a first questionnaire focusing on cardiovascular co-morbidities. A further questionnaire on sleep apnea syndrome (SAS) was delivered by the COM.E.T.A. study group to 107 endocrine centers in Italy. The results of our survey suggest that SAS is a well-known comorbidity even if its estimated prevalence is lower than in the literature. Polysomnography is the preferred tool for diagnosis. Control of SAS is considered relevant both for quality of life and co-morbidities. Continuous positive airway pressure is the cornerstone of therapy, but patients' acceptance may be critical. Control of GH/IGF-I secretion is important to improve SAS. Management of SAS requires cooperation between specialists.

  17. Diagnostic challenges and management of a patient with acromegaly due to ectopic growth hormone-releasing hormone secretion from a bronchial carcinoid tumour

    Kyriakakis, Nikolaos; Trouillas, Jacqueline; Dang, Mary N; Lynch, Julie; Belchetz, Paul; Korbonits, Márta

    2017-01-01

    Summary A male patient presented at the age of 30 with classic clinical features of acromegaly and was found to have elevated growth hormone levels, not suppressing during an oral glucose tolerance test. His acromegaly was originally considered to be of pituitary origin, based on a CT scan, which was interpreted as showing a pituitary macroadenoma. Despite two trans-sphenoidal surgeries, cranial radiotherapy and periods of treatment with bromocriptine and octreotide, his acromegaly remained active clinically and biochemically. A lung mass was discovered incidentally on a chest X-ray performed as part of a routine pre-assessment for spinal surgery 5 years following the initial presentation. This was confirmed to be a bronchial carcinoid tumour, which was strongly positive for growth hormone-releasing hormone (GHRH) and somatostatin receptor type 2 by immunohistochemistry. The re-examination of the pituitary specimens asserted the diagnosis of pituitary GH hyperplasia. Complete resolution of the patient’s acromegaly was achieved following right lower and middle lobectomy. Seventeen years following the successful resection of the bronchial carcinoid tumour the patient remains under annual endocrine follow-up for monitoring of the hypopituitarism he developed after the original interventions to his pituitary gland, while there has been no evidence of active acromegaly or recurrence of the carcinoid tumour. Ectopic acromegaly is extremely rare, accounting for <1% of all cases of acromegaly. Our case highlights the diagnostic challenges differentiating between ectopic acromegaly and acromegaly of pituitary origin and emphasises the importance of avoiding unnecessary pituitary surgery and radiotherapy. The role of laboratory investigations, imaging and histology as diagnostic tools is discussed. Learning points: Ectopic acromegaly is rare, accounting for less than 1% of all cases of acromegaly. Ectopic acromegaly is almost always due to extra-pituitary GHRH secretion

  18. Can immediate postoperative random growth hormone levels predict long-term cure in patients with acromegaly?

    Pinaki Dutta

    2016-01-01

    Conclusion: Early postoperative GH values may be used to predict long-term cure. A value of ≤1.5 ng/ml at 6 h following surgery may predict long-term cure in two-thirds of the patients with acromegaly who undergo TSS.

  19. A missed diagnosis of acromegaly during a female-to-male gender transition

    Roerink, S.; Marsman, D.; Bon, A. van; Netea-Maier, R.T.

    2014-01-01

    We present a case of a 46-year-old transgender male who, during his female-to-male transition, presented with a pituitary apoplexy at the emergency department of a general hospital in the Netherlands. During admission, it turned out that he also suffered from acromegaly due to a growth hormone secre

  20. Relationship between plasma growth hormone concentration and cellular sodium transport in acromegaly

    Herlitz, H.; Jonsson, O.; Bengtsson, B.-Aa. (Departments of Nephrology, Urology and Endocrinology, University of Goeteborg, Goeteborg (Sweden))

    1992-01-01

    We investigated the relationship between mean plasma growth hormone (GH) concentration and cellular sodium transport in untreated and treated acromegaly. Seventeen patients (age 55 [+-] 3 years) with active acromegaly were studied with respect to plasma GH (mean of 24 h GH profile) and erythrocyte electrolyte content as well as transmembrane sodium transport. The patients were reinvestigated two weeks after successful surgery (N = 14) and again after one year (N = 13). Erythrocyte electrolytes were analyzed by flame photometry and sodium influx and efflux rate constant determined by in vitro incubation using a modified Keyne's formula. In patients with active acromegaly there was a significant positive correlation between IGF-1 and cellular sodium transport, while GH tended to show a negative relatonship to the same parameter. After successful treatment, both IGF-1 and GH disclosed a positive relationship to cellular sodium transport. After one year, a significant increase in erythrocyte sodium content was seen in the patients compared to the preoperative situation. In conclusion, if this is a generalized phenomonen the results are compatible with a sodium-retaining effect of GH via stimulation of transmembrane sodium transport. In active acromegaly this may be counteracted by a sodium transport inhibitor giving the reverse relationship between GH and cellular sodium transport. (au).

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

    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

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

    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.

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

    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.

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

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

    2004-12-01

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

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

    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.

  6. Atrial conduction times and left atrium mechanical functions in patients with active acromegaly.

    Ilter, A; Kırış, A; Kaplan, Ş; Kutlu, M; Şahin, M; Erem, C; Civan, N; Kangül, F

    2015-03-01

    The aim of this study was to evaluate atrial electromechanical delay (EMD), P wave dispersion (Pwd), and left atrial (LA) mechanical functions in patients with active acromegaly. Twenty-three patients with active acromegaly and 27 age- and sex-matched controls were included in this study. All atrial electromechanical interval parameters (PA lateral, PA septum, PA tricuspid, interatrial EMD, intra-LA EMD, and intra-right atrial EMD) were measured from mitral lateral annulus, mitral septal annulus, and right ventricular tricuspid annulus by tissue Doppler imaging. LA volumes were measured by the disk method in the apical four-chamber view and were indexed to the body surface area. Mechanical function parameters of LA were calculated. Pwd was performed by 12-lead electrocardiograms. Atrial electromechanical intervals (PA lateral, PA septum, PA tricuspid, interatrial EMD, intra-LA EMD, and intra-right atrial EMD) and Pwd were similar between patients with acromegaly and control subjects (all p > 0.05). LA volumes (maximum, minimum, and presystolic) and LA mechanical functions were not significantly different between the groups (all p > 0.05). Additionally, serum levels of growth hormone and insulin-like growth factor-1 were not correlated with atrial electromechanical parameters and LA mechanical functions. Atrial electrical conduction times were not prolonged and LA mechanical functions were not impaired in patients with active acromegaly compared with controls. And the prevalence of supraventricular arrhythmia risk may not increase in this population.

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

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

    2011-01-01

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

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

    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.

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

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

    2016-01-01

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

  10. Active postoperative acromegaly: sustained remission after discontinuation of somatostatin analogues

    Cardenas-Salas, Jersy

    2016-01-01

    Summary 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. Learning points: The probability of disease recurrence is high after discontinuation of treatment with somatostatin analogues. Current data indicate that remission after treatment discontinuation may be more likely in patients with low GH and IGF1 levels before treatment withdrawal, in those who respond well to low-dose treatment, in those without evidence of adenoma on MRI, and/or in patients receiving prolonged treatment. This case report suggests that prolonged treatment with somatostatin analogues can be curative in carefully selected patients. PMID:27933171

  11. Bone mineral density and circulating cytokines in patients with acromegaly.

    Longobardi, S; Di Somma, C; Di Rella, F; Angelillo, N; Ferone, D; Colao, A; Merola, B; Lombardi, G

    1998-11-01

    Acromegalic patients present an increase of osteoblastic and osteoclastic activity, showing a different effect on the axial and appendicular skeletal structures. At this regard controversial data about bone mineral density (BMD) have been published in literature. In fact an increase of BMD levels in femoral neck and Ward's triangle without any difference in lumbar spine has been described. On the other hand normal BMD levels at forearm and reduced BMD levels at lumbar spine were found. These patients seem to have a reduction of trabecular BMD similar to postmenopausal osteoporotic patients despite normal or slightly elevated cortical BMD. Recently, it has been described that cytokines, in particular tumor necrosis factor-alpha (TNF-alpha) and interleukin-1 (IL-1), are implicated in the pathogenetic mechanism of postmenopausal osteoporosis. Taking into account that growth hormone (GH) can increase TNF-alpha and IL-1 secretion by mononuclear blood cells, the evaluation of possible relationship between the reduced BMD at lumbar spine and circulating cytokines levels was carried out in acromegalic patients. In addition we evaluated the effect of acute octreotide administration on serum TNF-alpha and IL-I concentrations. Eleven patients with active acromegaly and eleven healthy age-, sex-, weight- and heightmatched subjects were enrolled in this study. BMD was significantly reduced at lumbar spine (0.80 +/- 0.29 g/cm2 vs 1.02 +/- 0.11 g/cm2; p affect bone turnover inducing an increase of cytokines acting by a paracrine/autocrine mechanism cannot be ruled out.

  12. Gigantism and Acromegaly Due to Xq26 Microduplications and GPR101 Mutation

    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. Lanreotide autogel(®): a review of its use in the treatment of patients with acromegaly.

    Burness, Celeste B; Dhillon, Sohita; Keam, Susan J

    2014-09-01

    Lanreotide Autogel(®) (ATG) [Somatuline(®) Autogel(®), Somatuline(®) Depot(®)] is a prolonged-release, supersaturated aqueous gel formulation of the somatostatin analogue lanreotide acetate that acts via somatostatin receptors to reduce both growth hormone and insulin-like growth factor-I levels. It is indicated for the treatment of patients with acromegaly who have had an inadequate response to or cannot be treated with surgery and/or radiotherapy. This article reviews the clinical efficacy and tolerability of lanreotide ATG in the treatment of acromegaly, as well as summarizing its pharmacological properties. Results of clinical trials and extension studies of up to 4 years duration showed that deep subcutaneous lanreotide ATG was a generally effective treatment in treatment-naive and treatment-experienced adults with acromegaly. Lanreotide ATG provided hormonal control and improved both health-related quality of life and acromegaly symptoms in most patients; it also reduced tumour volume to a clinically significant extent in studies of primary therapy. Moreover, lanreotide ATG was generally no less effective than intramuscular lanreotide long-acting microparticles and was as effective as intramuscular octreotide long-acting release in switching or crossover studies, including those with standard or extended dosing intervals. Lanreotide ATG is generally well tolerated; the most frequently reported adverse events were mild or moderate transient gastrointestinal symptoms. Lanreotide ATG also has the advantage of being available in a convenient pre-filled syringe and is given subcutaneously rather than intramuscularly. Thus, lanreotide ATG continues to be a valuable option in the treatment of acromegaly, with potential advantages being ease of administration and longer dosing intervals in patients who have an adequate response to initial therapy.

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

    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...

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

    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...

  16. Valve-sparing aortic root replacement and aortic valve repair in a patient with acromegaly and aortic root dilatation

    Karel Van Praet

    2015-07-01

    Full Text Available Aortic regurgitation and dilatation of the aortic root and ascending aorta are severe complications of acromegaly. The current trend for management of an aortic root aneurysm is valve-sparing root replacement as well as restoring the diameter of the aortic sinotubular junction (STJ and annulus. Our case report supports the recommendation that in patients with acromegaly, severe aortic root involvement may indicate the need for surgery.

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

    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...

  18. Clinical features and therapeutic outcomes of acromegaly during the recent 10 years in a single institution in Japan.

    Fukuda, Izumi; Hizuka, Naomi; Muraoka, Toko; Kurimoto, Makiko; Yamakado, Yu; Takano, Kazue; Ichihara, Atsuhiro

    2014-02-01

    Untreated acromegaly is associated with a twofold to fourfold increased mortality risk compared to the population. Recently, new therapeutic modalities have been developed and may contribute to an improvement in treatment outcomes in patients with acromegaly. In the current study we determined the clinical features and recent therapeutic outcomes in patients with acromegaly. The initial symptoms, selected therapeutic modalities, and outcomes in 125 patients with acromegaly (M/F, 49/76, 19-86 years) who were admitted to our institution between 2001 and 2010 were analyzed using medical charts. The basal GH levels and IGF-I SD scores in the patients ranged from 0.17 to 90.21 μg/L and 1.9-13.6, respectively. Acral enlargement (face, hands, and feet) without overt complications was essential to the diagnosis in 49 % of the patients. In these cases, it required 5 years to establish the diagnosis of acromegaly after symptom onset. Twenty (16 %) and 13 (10 %) patients had diabetes mellitus and hypertension 6 years prior to the diagnosis of acromegaly, respectively. In 35 patients with microadenomas, the rate of controlled cases following transsphenoidal surgery was 93 %. In 90 patients with macroadenomas, the remission rate was 79 % with multidisciplinary treatment. In cases in which the tumor extended beyond the lateral tangent of the internal carotid artery (Knosp grade ≥3), the remission rate was 33-56 %. Improvements in surgical techniques and medical therapies may contribute to increased rates of controlled cases in patients with acromegaly, although advanced lateral extension of the tumor remains a critical determinant of the therapeutic outcome.

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

    Masahiro Iikubo

    2012-01-01

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

  20. Morphological and histopathological changes in orofacial structures of experimentally developed acromegaly-like rats: an overview.

    Iikubo, Masahiro; Kojima, Ikuho; Sakamoto, Maya; Kobayashi, Akane; Ikeda, Hidetoshi; Sasano, Takashi

    2012-01-01

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

  1. Reduction of elevated IGF-1 levels in coincident amyotrophic lateral sclerosis and acromegaly.

    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.

  2. Pharmacological activation of the GABAergic system does not affect GH and PRL release in acromegaly.

    Orio, F; Iovino, M; Monteleone, P; Agrusta, M; Steardo, L; Lombardi, G

    1988-11-01

    An extensive hypothalamic neurotransmitter impairment has been proposed in acromegaly. However, at the moment, the hypothalamic GABAergic system has been little investigated in this disorder. Since GABA has been shown to modulate growth hormone (GH) and prolactin (PRL) secretion in human subjects, it seemed reasonable to investigate hypothalamic GABAergic functioning through the assessment of basal GH and PRL responses to pharmacological activation of this system. 800 mg of sodium valproate (SV), a drug with GABA facilitating properties, were administered orally to 7 acromegalic patients and 9 healthy volunteers. Blood samples were collected before and after the drug administration for the measurement of plasma GH and PRL levels. SV induced a clear-cut rise in basal GH and a decrease in basal PRL in healthy subjects, but it did not induce any change in the basal levels of these hormones in acromegalics. These results suggest that the response of GH and PRL to SV in acromegaly is qualitatively different from normal controls.

  3. Surgery and Radiosurgery for Acromegaly: A Review of Indications, Operative Techniques, Outcomes, and Complications

    Yvette Marquez

    2012-01-01

    Full Text Available Among multimodality treatments for acromegaly, the goals of surgical intervention are to balance maximal tumor resection while preserving normal pituitary function and maintaining patient safety. The resection of growth hormone-(GH- secreting pituitary adenomas in the hands of experienced surgeons results in hormonal remission in 50–70% of patients. Acromegalic patients often have medical comorbidities and anatomical variations complicating anesthesia and surgical management. Despite these challenges, complications such as CSF leak or new hypopituitarism following surgery remain uncommon. Over the past decade, endoscopic approaches to pituitary tumors have improved visualization and facilitated identification of additional tumor using angled telescopes. Patients with persistent acromegaly following surgery require continued medical and/or radiation-based interventions. The adjunctive use of stereotactic radiosurgery offers hormonal remission in 40–50% of patients. In this article, the current preoperative evaluation, indications for surgery, surgical approaches, role of radiosurgery, complications, and remission criteria following operative resection of GH adenomas are reviewed.

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

    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.

  5. Efficiency and safety of OctreotidLong FS therapy in acromegaly patients

    A V Dreval’

    2013-03-01

    Full Text Available Aim of this study was to investigate efficiency and safety of OctreotidLong FS in patients with acromegaly. Materials and methods. 41 patients with acromegaly (8 – de novo and 33 patients after different somato statin analogs treatment was treated OctreotidLong FS one injection in 28 days. Growth hormone (GH, Insulin like Growth Factor 1 (IFG1, fasting glucose (FG and HbA1c were assess after 3, 6 and 12 month of therapy. Results. We found out the decreasing of GH and IGF1 from 12,8 (8,0–82,7 mU/ml to 3,8 (1,6–13,8 mU/ml ( p < 0,05 and %IGF1 increasing (% IGF1 from 231 (150–286% to 9,5 (−26–111% ( p < 0,05 in 8 de novo acromegalic patients. We also revealed that IGF1 didn’t change and GH decreased after 3 month (33 patients, 6 month (22 patients and 12 month (8 patients of OctreotidLong FS treatment. We didn’t observed negative effect of OctreotidLong FS treatment to carbohydrate metabolism in patients with acromegaly. Conclusion. The therapy of OctreotidLong FS leads to induce successful control of GH and IGFI in 50% de novo patients and didn’t change the number of patients with control of acromegaly after another somato statin analogs treatment. Carbohydrate metabolism also didn’t change after OctreotidLong FS treatment.

  6. Polycythemia as rare secondary direct manifestation of acromegaly: management and single-centre epidemiological data.

    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.

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

    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.

  8. Communication practices and awareness of resources for acromegaly patients among endocrinologists

    Polanco-Briceno, Susan; Glass, Daniel; Plunkett, Cindy

    2016-01-01

    Purpose This study was designed to assess the awareness and utilization of resources to improve patients’ treatment experiences among endocrinologists who currently treat patients with acromegaly. Methods A total of 4,280 US endocrinologists were randomly selected from the CMS National Plan and Provider Enumeration System and were invited by mail to participate in a 20-minute online survey. In order to qualify, respondents had to be the primary physician making treatment decisions for at least one patient for their acromegaly. Results Results are based on responses from 126 physicians from primarily urban and suburban practices, with a median of five acromegaly patients. A total of 70% of patients are currently receiving drug therapy; among these, 91% are on octreotide (51%), lanreotide (29%), or pasireotide (11%), alone or in combination with another therapy. Nearly half of the respondents thought that the impact of patient adherence on therapy outcome for acromegaly was either not very (40%) or not at all (7%) significant. Respondents who believe patient adherence significantly impacts treatment outcome were significantly more likely to discuss automated adherence reminders (50% vs 26%; P=0.015), mobile administration programs (57% vs 35%; P=0.029), and symptom tracking (72% vs 42%; P=0.002). Overall, 44% of respondents routinely recommend education/emotional support programs, and 25% routinely recommend financial assistance programs. Respondents who believe patient adherence significantly impacts treatment outcome generally were more familiar with individual education and emotional support programs compared to those who do not, although they were not more likely to routinely refer patients to any of these resources. Conclusion There are unmet needs with respect to increasing awareness among physicians of the importance of patient adherence to therapy, resources available to patients, and how collaboration among patients, nurses, and physicians can improve

  9. Molecular biological examination of somatotroph pituitary adenomas related to clinical data from patients with acromegaly

    2010-01-01

    Definition and epidemiology The first historical description of acromegaly could be the story of David and Goliath described both in The Old Testament and in the Koran. Goliath was a giant, but David defeated him by sneaking up on him, maybe in his visual field defect, and hitting him with a stone in the forehead using a sling. The connection between gigantism and the pituitary, hence with the possibility of visual disturbances, was not recognized until 1884, published in a book by Fritzsc...

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

    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

  11. Increased Short-Term Beat-To-Beat Variability of QT Interval in Patients with Acromegaly.

    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.

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

    Plunkett C

    2015-07-01

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

  13. Attitudes and preferences in patients with acromegaly on long-term treatment with somatostatin analogues

    Cecilia Follin

    2016-08-01

    Full Text Available Introduction: Patients with acromegaly can be treated with surgery, medical therapy and/or radiation therapy. For the patients not being cured with surgery, treatment with somatostatin analogues (SSAs is the primary therapy. SSA can be taken by self- or partner-administered injections in addition to being given by a nurse at a clinic. The aim was to assess if patients with acromegaly prefer self-injections and to investigate their attitudes towards long-term medical therapy. Method: All patients in the southern medical region of Sweden with a diagnosis of acromegaly and treated with SSA were eligible for the study (n = 24. The study is based on a questionnaire asking about the patients’ attitudes and preferences for injections with SSA, including their attitudes towards self-injection with SSA. Results: The patients’ (23 included median age was 68.5 years and the patients had been treated with SSA for 13 (1–38 years. One patient was currently self-injecting. All of the other patients were receiving injections from a nurse at a clinic. Three patients preferred self-injections, one preferred partner injections and 19 patients did not prefer self- or partner injections. The most frequent arguments to not preferring self-injections were ‘feeling more secure with an educated nurse’ and ‘preferring regular contact with a specialised nurse’. Conclusion: Patients with acromegaly prefer regular contact with the endocrine team to the independence offered by self-injections. These findings might mirror the patients’ desires for continuity and safety. We need to address patients’ concerns regarding injections with SSA and support them in their choices.

  14. Increased Population Risk of AIP‐Related Acromegaly and Gigantism in Ireland

    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

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

    Purnima Agarwal

    2016-01-01

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

  16. Acromegaly presenting with low insulin-like growth factor-1 levels and diabetes: a case report

    Wijayaratne, Dilushi Rowena; Arambewela, M. H.; Dalugama, Chamara; Wijesundera, Dishni; Somasundaram, Noel; Katulanda, Prasad

    2015-01-01

    Introduction Acromegaly is an endocrine disorder arising from excessive serum growth hormone levels in adulthood and is characterized by progressive somatic enlargement. Biochemical confirmation is achieved by demonstration of elevated baseline serum growth hormone levels which are not suppressed during an oral glucose tolerance test, and by increased levels of serum insulin-like growth factor-1. The serum insulin-like growth factor-1 level provides an assessment of integrated growth hormone ...

  17. Spontaneous acromegaly: a retrospective case control study in German shepherd dogs.

    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.

  18. 5 Long-term acromegaly and associated cardiovascular complications: a case-based review.

    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.

  19. Contribution of gluconeogenesis and glycogenolysis to hepatic glucose production in acromegaly before and after pituitary microsurgery.

    Höybye, C; Chandramouli, V; Efendic, S; Hulting, A-L; Landau, B R; Schumann, W C; Wajngot, A

    2008-07-01

    The diabetogenic effect of excess growth hormone (GH) such as that in acromegaly is well known. However, the contribution of the various components to hepatic glucose production (HGP) is not completely understood. In this study we evaluated insulin resistance, HGP, gluconeogenesis (GNG), and glycogenolysis (GLY) in five patients with acromegaly before and after pituitary microsurgery. Insulin resistance was estimated by the HOMA index. HGP was measured using a primed continuous (6,6- 2H2) glucose infusion, and GNG was measured from 2 H enrichment at carbons 2 and 5 of blood glucose on ingestion of 2H2O. The ratio of these enrichments equals the fractional contribution of GNG to HGP, and GLY was calculated as the difference between HGP and GNG. All measurements were performed after 12 hours of fasting. Levels of GH and IGF-I decreased, as did the HOMA index (p<0.05). HGP was reduced from 11.4 micromol/kg/min to 9.7 micromol/kg/min (p=0.032). GNG contributed most to HGP. GNG was unchanged, whereas GLY's fraction decreased 29% (p=0.056) postoperatively. This pilot study indicates that GNG is the main contributor to HGP and that GLY is more sensitive than is GNG to the insulin resistance existing in acromegaly.

  20. Stimulatory and inhibitory effects of neuropeptide Y on growth hormone secretion in acromegaly in vivo.

    Watanobe, H; Tamura, T

    1997-02-01

    It has been reported that neuropeptide Y (NPY) affects growth hormone (GH) secretion in several animal species. With respect to the role of NPY in regulating GH release in humans, one previous study has reported that NPY inhibited GH secretion from cultured GH-secreting pituitary adenoma cells in vitro. However, since it has yet to be explored whether NPY affects GH secretion in acromegaly in vivo, in this study we attempted to examine the effect of intravenous (i.v.) bolus injection of 100 microg of human NPY on plasma GH levels in 15 patients with active acromegaly, trying to find a possible correlation among GH responses to NPY, thyrotropin-releasing hormone (TRH;500 microg, i.v.), luteinizing hormone-releasing hormone (LHRH;100 microg, i.v.), and bromocriptine (Br;2.5 mg, per os). NPY significantly increased GH secretion (more than twice the basal level) in 4 (27%) patients, and all of them were responsive to LHRH and non-responsive to Br. In contrast, 3 (20%) acromegalics showed a significant decrease in GH levels (less than half the baseline) after NPY, and all these patients were responsive to both TRH and Br. From these results, we hypothesize that the NPY-induced increase in GH release may be a feature of somatotroph-like pituitary adenoma causing acromegaly, whereas the NPY-induced decrease in GH secretion may be a feature of lactotroph-like adenoma.

  1. [Suppression by dopamine of GH release induced by GRF in a case of acromegaly].

    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.

  2. Primary treatment of acromegaly with high-dose lanreotide: a case series

    Cordes Uwe

    2010-03-01

    Full Text Available Abstract Introduction The first-line treatment for acromegaly is transsphenoidal surgery. In approximately 50% of patients, however, a cure is not possible with surgery and alternatives are needed. Somatostatin analog therapy is the recommended first-line treatment in patients with such cases. Here we provide the first report of a high-dose lanreotide primary therapy in patients with acromegaly. Case presentation Six patients who were not suitable for surgery were given 60 mg of lanreotide (Autogel® every four weeks. All patients were German nationals and Caucasian. When the response of our patients was unsatisfactory, the dose was increased sequentially to 90 mg every four weeks, 120 mg every four weeks, 120 mg every three weeks and 180 mg every three weeks. Treatment duration was 12 to 24 months. In all cases, the lanreotide dose was 120 mg every 4 weeks or higher. In five of our patients, growth hormone (GH levels were successfully reduced (in three patients GH Tumor shrinkage or degeneration was observed in the five responding patients. No drug-related adverse events were noted. Conclusions These results suggest that lanreotide at high doses of 120 mg every four weeks or more is an effective first-line therapy for patients with acromegaly that surgery alone cannot treat.

  3. A risk-benefit assessment of octreotide in the treatment of acromegaly.

    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.

  4. ["Die grosse Barb" in the museum of the University of Marburg. An early documentation of acromegaly].

    Krause, W; Rassner, G; Happle, R

    2009-06-01

    The university museum for cultural history in the castle of Marburg has a portrait "Die grosse Barb", which represents a women suffering from acromegaly. She shows the typical pathologic alterations: thickening of the skin folds, thickening of the lips and the eyelids, growth of bones and cartilages, lengthening of the nose, enlargement of the ears, protrusion of the zygoma, mandible and the chin. Acromegaly is a consequence of enhanced secretion of growth hormone, which occurs also as a symptom of several syndromes, such as multiple endocrine neoplasia type 1, McCune-Albright-syndrome, and NAME syndrome (Carney complex type I). The most remarkable symptom of acromegaly is the gigantism. This occurs also in androgen-deficient states, such as the Klinefelter syndrome and some more genetic syndromes, of which the Simpson-Golabi-Behmel syndrome, the Sotos syndrome, the Marfan syndrome, the homocystinuria, and the fragile X-syndrome may be mentioned. Nothing is known on the further fate of the patient shown in the portrait. It is also unknown, whether she owes her position as a chambermaid to her gigantism, for it was a common use in courts to have people with abnormal body shapes in attendance.

  5. 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).

    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.

  6. A case for hypothalamic acromegaly: a clinicopathological study of six patients with hypothalamic gangliocytomas producing growth hormone-releasing factor.

    Asa, S L; Scheithauer, B W; Bilbao, J M; Horvath, E; Ryan, N; Kovacs, K; Randall, R V; Laws, E R; Singer, W; Linfoot, J A

    1984-05-01

    We report the histological, ultrastructural, and immunocytochemical features of six hypothalamic gangliocytomas associated with pituitary GH cell adenomas and/or acromegaly. In four patients, the gangliocytoma was intrasellar, and no hypothalamic investigation was performed; in two patients, autopsy confirmed hypothalamic involvement. Four patients had a gangliocytoma associated with pituitary GH cell adenoma and acromegaly; electron microscopy demonstrated an intimate association between neurons and adenomatous GH cells. One patient had a gangliocytoma and a GH cell adenoma but no clinical evidence of acromegaly. In the sixth patient, clinical and biochemical acromegaly was manifest, but no pituitary adenoma was demonstrated. Using immunocytochemistry, human pancreatic tumor GRF (hptGRF-40) was localized in the majority of neurons of all six gangliocytomas. The pituitary adenomas and nontumorous adenohypophyses were negative for hptGRF-40. In addition, somatostatin, glucagon, and GnRH were demonstrated within some neurons of several tumors; insulin and gastrin stains were equivocal. These findings confirm previous proposals of production of a GRF by such gangliocytomas. While the significance of other peptides found in some of the tumors is uncertain, the presence of hptGRF-40 in neurons of these gangliocytomas supports the theory that GRF excess is the mechanism responsible for over-production of GH and provides evidence for a syndrome of hypothalamic acromegaly.

  7. High-resolution-cone beam tomography analysis of bone microarchitecture in patients with acromegaly and radiological vertebral fractures.

    Maffezzoni, Filippo; Maddalo, Michele; Frara, Stefano; Mezzone, Monica; Zorza, Ivan; Baruffaldi, Fabio; Doglietto, Francesco; Mazziotti, Gherardo; Maroldi, Roberto; Giustina, Andrea

    2016-11-01

    Vertebral fractures are an emerging complication of acromegaly but their prediction is still difficult occurring even in patients with normal bone mineral density. In this study we evaluated the ability of high-resolution cone-beam computed tomography to provide information on skeletal abnormalities associated with vertebral fractures in acromegaly. 40 patients (24 females, 16 males; median age 57 years, range 25-72) and 21 healthy volunteers (10 females, 11 males; median age 60 years, range: 25-68) were evaluated for trabecular (bone volume/trabecular volume ratio, mean trabecular separation, and mean trabecular thickness) and cortical (thickness and porosity) parameters at distal radius using a high-resolution cone-beam computed tomography system. All acromegaly patients were evaluated for morphometric vertebral fractures and for mineral bone density by dual-energy X-ray absorptiometry at lumbar spine, total hip, femoral neck, and distal radius. Acromegaly patients with vertebral fractures (15 cases) had significantly (p cone-beam computed tomography at the distal radius may be useful to evaluate and predict the effects of acromegaly on bone microstructure.

  8. Increased Short-Term Beat-To-Beat Variability of QT Interval in Patients with Acromegaly.

    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

  9. Low Prevalence of Periodontitis in Acromegaly: Growth Hormone May Exert a Protective Effect

    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

  10. Efficacy, safety, and pharmacokinetics of sustained-release lanreotide (lanreotide Autogel) in Japanese patients with acromegaly or pituitary gigantism.

    Shimatsu, Akira; Teramoto, Akira; Hizuka, Naomi; Kitai, Kazuo; Ramis, Joaquim; Chihara, Kazuo

    2013-01-01

    The somatostatin analog lanreotide Autogel has proven to be efficacious for treating acromegaly in international studies and in clinical practices around the world. However, its efficacy in Japanese patients has not been extensively evaluated. We examined the dose-response relationship and long-term efficacy and safety in Japanese patients with acromegaly or pituitary gigantism. In an open-label, parallel-group, dose-response study, 32 patients (29 with acromegaly, 3 with pituitary gigantism) received 5 injections of 60, 90, or 120 mg of lanreotide Autogel over 24 weeks. Four weeks after the first injection, 41% of patients achieved serum GH level of gigantism) received lanreotide Autogel once every 4 weeks for a total of 13 injections. Dosing was initiated with 90 mg and adjusted according to clinical responses at Weeks 16 and/or 32. At Week 52, 47% of patients had serum GH levels of gigantism.

  11. Bone mineral density of lumbar spine and femur in acromegaly. Knochendichte an Lendenwirbelsaeule und Femur bei Akromegalie

    Huebsch, P. (Ludwig-Boltzmann-Institut fuer Radiologisch-Physikalische Tumordiagnostik, Vienna (Austria)); Kotzmann, H. (Universitaetsklinik fuer Innere Medizin 3, Vienna (Austria)); Svoboda, T. (Universitaetsklinik fuer Innere Medizin 3, Vienna (Austria)); Kainberger, F.M. (Ludwig-Boltzmann-Institut fuer Radiologisch-Physikalische Tumordiagnostik, Vienna (Austria)); Bankier, A. (Ludwig-Boltzmann-Institut fuer Radiologisch-Physikalische Tumordiagnostik, Vienna (Austria)); Seidl, G. (Ludwig-Boltzmann-Institut fuer Radiologisch-Physikalische Tumordiagnostik, Vienna (Austria))

    1993-08-01

    Acromegaly is regarded as a cause for secondary osteoporosis, whereas recent papers suggest that growth hormone increases bone mineral density (BMD). In 16 patients with active acromegaly we found an increased BMD compared to normal controls in the lumbar spine and the proximal femur by means of dual energy X-ray absoptiometry. This increase in BMD was statistically significant in the femoral neck and in Ward's triangle (P=0.05). Moreover, no signs of osteoporosis were found radiologically. (orig.)

  12. Growth hormone modulation of arginine-induced glucagon release: studies of isolated growth hormone deficiency and acromegaly.

    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.

  13. Diagnostic challenges and management of a patient with acromegaly due to ectopic growth hormone-releasing hormone secretion from a bronchial carcinoid tumour

    Nikolaos Kyriakakis

    2017-01-01

    Full Text Available A male patient presented at the age of 30 with classic clinical features of acromegaly and was found to have elevated growth hormone levels, not suppressing during an oral glucose tolerance test. His acromegaly was originally considered to be of pituitary origin, based on a CT scan, which was interpreted as showing a pituitary macroadenoma. Despite two trans-sphenoidal surgeries, cranial radiotherapy and periods of treatment with bromocriptine and octreotide, his acromegaly remained active clinically and biochemically. A lung mass was discovered incidentally on a chest X-ray performed as part of a routine pre-assessment for spinal surgery 5 years following the initial presentation. This was confirmed to be a bronchial carcinoid tumour, which was strongly positive for growth hormone-releasing hormone (GHRH and somatostatin receptor type 2 by immunohistochemistry. The re-examination of the pituitary specimens asserted the diagnosis of pituitary GH hyperplasia. Complete resolution of the patient’s acromegaly was achieved following right lower and middle lobectomy. Seventeen years following the successful resection of the bronchial carcinoid tumour the patient remains under annual endocrine follow-up for monitoring of the hypopituitarism he developed after the original interventions to his pituitary gland, while there has been no evidence of active acromegaly or recurrence of the carcinoid tumour. Ectopic acromegaly is extremely rare, accounting for <1% of all cases of acromegaly. Our case highlights the diagnostic challenges differentiating between ectopic acromegaly and acromegaly of pituitary origin and emphasises the importance of avoiding unnecessary pituitary surgery and radiotherapy. The role of laboratory investigations, imaging and histology as diagnostic tools is discussed.

  14. Impaired decision making and delayed memory are related with anxiety and depressive symptoms in acromegaly.

    Crespo, Iris; Santos, Alicia; Valassi, Elena; Pires, Patricia; Webb, Susan M; Resmini, Eugenia

    2015-12-01

    Evaluation of cognitive function in acromegaly has revealed contradictory findings; some studies report normal cognition in patients with long-term cured acromegaly, while others show attention and memory deficits. Moreover, the presence of affective disorders in these patients is common. Our aim was to evaluate memory and decision making in acromegalic patients and explore their relationship with affective disorders like anxiety and depressive symptoms. Thirty-one patients with acromegaly (mean age 49.5 ± 8.5 years, 14 females and 17 males) and thirty-one healthy controls participated in this study. The Iowa Gambling Task (IGT), Rey Auditory Verbal Learning Test, State-Trait Anxiety Inventory, and Beck Depression Inventory-II (BDI-II) were used to evaluate decision making, verbal memory, anxiety, and depressive symptoms, respectively. Acromegalic patients showed impairments in delayed verbal memory (p < 0.05) and more anxiety and depressive symptoms (p < 0.05) than controls. In the IGT, acromegalic patients presented an altered decision-making strategy compared to controls, choosing a lower number of the safer cards (p < 0.05) and higher number of the riskier cards (p < 0.05). Moreover, multiple correlations between anxiety and depressive symptoms and performance in memory and decision making were found. Impaired delayed memory and decision making observed in acromegalic patients are related to anxiety and depressive symptoms. Providing emotional support to the patients could improve their cognitive function. A key clinical application of this research is the finding that depressive symptoms and anxiety are essentially modifiable factors.

  15. Health outcomes in acromegaly: depression and anxiety are promising targets for improving reduced quality of life

    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.

  16. Optimizing control of acromegaly: integrating a growth hormone receptor antagonist into the treatment algorithm.

    Clemmons, David R; Chihara, Kazuo; Freda, Pamela U; Ho, Ken K Y; Klibanski, Anne; Melmed, Shlomo; Shalet, Stephen M; Strasburger, Christian J; Trainer, Peter J; Thorner, Michael O

    2003-10-01

    Acromegaly is associated with significant morbidities and a 2- to 3-fold increase in mortality because of the excessive metabolic action of GH and IGF-I, a marker of GH output. Reductions in morbidity correspond with decreases in IGF-I, and mortality is lowered following normalization of IGF-I or GH levels. Therefore, this has become an important end point. Current guidelines for the treatment of acromegaly have not considered recent advances in medical therapy, in particular, the place of pegvisomant, a GH receptor antagonist. Treatment goals include normalizing biochemical markers, controlling tumor mass, preserving pituitary function, and relieving signs and symptoms. Surgery reduces tumor volume and is considered first-line therapy. Radiation reduces tumor volume and GH and IGF-I levels, but the onset of action is slow and hypopituitarism typically develops. Therefore, pharmacotherapy is often used following surgery or as first-line therapy for nonresectable tumors. Dopamine agonists can be considered in patients exhibiting minimal disease or those with GH-prolactin-cosecreting tumors but will not achieve hormone normalization in most patients. Somatostatin analogs effectively suppress GH and IGF-I in most patients, but intolerance (e.g. diarrhea, cramping, gallstones) can occur. Pegvisomant, the newest therapeutic option, blocks GH action at peripheral receptors, normalizes IGF-I levels, reduces signs and symptoms, and corrects metabolic defects. Pegvisomant does not appear to affect tumor size and has few adverse effects. Pegvisomant is the most effective drug treatment for acromegaly in normalizing IGF-I and producing a clinical response; it is the preferred agent in patients resistant to or intolerant of somatostatin analogs.

  17. Lack of rise in serum prolactin following yttrium-90 interstitial irradiation for acromegaly

    Clark, A.J.L.; Chahal, P.; Mashiter, K.; Joplin, G.F. (Royal Postgraduate Medical School, London (UK))

    1983-11-01

    The authors have investigated the possibility that the increase in serum PRL levels observed in patients with acromegaly treated with external irradiation could be due to damage to the hypothalamus or portal vessels, by comparing the effects of yttrium-90 interstitial irradiation, which is highly localised and does not normally extend to the hypothalamus, in a similar series of patients. These results are consistent with the hypothesis; a less likely explanation is that an overgrowth of radio-resistant PRL-secreting tumour cells is occurring after external irradiation, but not after yttrium-90 implantation.

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

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

    2007-01-01

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

  19. Clinical features and differential diagnosis of pituitary tumours with emphasis on acromegaly.

    Hennessey, J V; Jackson, I M

    1995-04-01

    Pituitary adenomas are frequently encountered, benign intracranial tumours. Clinically classified according to their capacity to produce and secrete hormones, pituitary tumours are diagnosed from the clinical manifestations and biochemical findings of specific pituitary hormone overproduction or of impaired pituitary function due to pressure on normal pituitary cells, the pituitary stalk or the hypothalamus. Additionally, the tumour may result in neurological manifestations due to its effect as an intracranial space-occupying lesion. Pituitary adenomas may present acutely with pituitary apoplexy after intrapituitary haemorrhage or infarction. The subsequent hypofunction of the pituitary with concomitant neurological sequelae of an expanding intracranial mass are often associated with excruciating headache, diplopia and visual field defects. Gradually developing neurological deficits or secondary endocrine failure over several years may precede the recognition of non-secretory tumours (30-40% of pituitary adenomas) as well as some of the hormone-producing adenomas, especially when they expand beyond the confines of the sella turcica. Asymptomatic masses occur in the pituitary in 5-27% of unselected autopsy series. About 10-20% of pituitaries imaged as part of a brain study contain lesions 'consistent with a pituitary adenoma', with about half being pituitary adenomas ('incidentalomas'). Many advocate screening such cases for a wide spectrum of pituitary function abnormalities. Clinical judgement should be utilized to determine the extent of the work-up and the frequency of follow-up. Acromegaly, a clinical syndrome caused by excess growth hormone secretion, accounts for one-sixth of resected pituitary tumours. This disorder leads to chronic progressive disability and a shortened life span, with approximately 50% of untreated acromegalic patients experiencing premature death. The prevalence of acromegaly has been estimated to range from 50 to 70 per million, with the

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

    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

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

    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. An uncommon case of dyspnea with unilateral laryngeal paralysis in acromegaly.

    Lerat, Justine; Lacoste, Marie; Prechoux, Jean-Marc; Aubry, Karine; Nadalon, Sylvie; Ly, Kim Heang; Bessede, Jean-Pierre

    2016-02-01

    A 61-year-old man with obstructive sleep apnea syndrome and normal BMI complained of dyspnea. Nasofibroscopy revealed a global and major oedema of the glottis and supraglottis and also a paralysis of the left vocal fold. CT-scan pointed out a spontaneous hyperdensity of the left arytenoid cartilage. A tracheostomy was performed. Clinical examination revealed large hands and macroglossy with high IGF1 rate. MRI confirmed a supracentimetric pituitary adenoma. To our knowledge, this is the first description of a case of acute respiratory distress due to unilateral larynx paralysis leading to acromegaly diagnosis. This is due to submucosal hypertrophy and vocal cord immobility.

  3. Ectopic acromegaly due to a growth hormone-secreting neuroendocrine-differentiated tumor developed from ovarian mature cystic teratoma.

    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.

  4. Ectopic Acromegaly Arising from a Pituitary Adenoma within the Bony Intersphenoid Septum of a Patient with Empty Sella Syndrome

    Arzamendi, Audrey E.; Shahlaie, Kiarash; Latchaw, Richard E.; Lechpammer, Mirna; Arzumanyan, Hasmik

    2016-01-01

    Objective  To describe the work-up and treatment of rare ectopic acromegaly caused by a biopsy-proven somatotroph pituitary adenoma located within the bony intersphenoid septum of a patient with empty sella syndrome (ESS). Methods  We report the presentation, clinical course, diagnostic work-up, and lesion localization and treatment challenges encountered in a 55-year-old patient, with a brief review of relevant literature. Results  A 55-year-old African-American man presented with acromegaly and ESS. Attempts to definitively localize the causative tumor were unsuccessful, though petrosal sinus sampling supported central growth hormone production and imaging suggested bone-enclosed subsellar pituitary tissue. Endoscopic endonasal transphenoidal exploration was undertaken with resection of a somatotroph pituitary microadenoma, and subsequent clinical improvement and biochemical remission. Retrospective review revealed the patient's pituitary to have been located ectopically within a unique bony intersphenoid septum. Conclusion  This report describes the first known case of an ectopic pituitary adenoma located within the midline bony intersphenoid septum, which we postulate to have resulted from anomalous embryological pituitary migration. Intra-intersphenoid septal tumors should be considered in cases of apparent central acromegaly with ESS or absence of tumor tissue within the paranasal sinuses or other peripheral locations. Indexing  Acromegaly, ESS, pituitary adenoma, sphenoid sinus septum. PMID:27468406

  5. Acromegaly: the significance of serum total and free IGF-I and IGF-binding protein-3 in diagnosis

    A-J. van der Lely (Aart-Jan); W.W. de Herder (Wouter); J.A.M.J.L. Janssen (Joop); S.W.J. Lamberts (Steven)

    1997-01-01

    textabstractWe have studied the physiological and clinical relevance of measurements of serum total and free IGF-I and IGF-binding protein-3 (IGFBP-3) in 57 previously untreated patients with active acromegaly (32 males, 25 females; mean age 47 years) as compared with sex- and age-

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

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

    2010-01-01

    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...

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

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

    2014-01-01

    CONTEXT: GH deficiency (GHD) may occur in adults with cured acromegaly (acroGHD). OBJECTIVE: Our objective was to examine the effectiveness and safety of GH replacement in acroGHD. DESIGN: This study was a retrospective analysis of data from KIMS (Pfizer International Metabolic Database). SETTING...

  8. Assessment of cardiac autonomic functions by heart rate recovery, heart rate variability and QT dynamicity parameters in patients with acromegaly.

    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

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

    Sreenan Seamus

    2012-09-01

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

  10. Acromegaly due to a Macroinvasive Plurihormonal Pituitary Adenoma and a Rectal Carcinoid Tumor

    Sang Ouk Chin

    2015-09-01

    Full Text Available A macroinvasive pituitary adenoma with plurihormonality usually causes acromegaly and hyperprolactinemia, and also accompanies with neurologic symptoms such as visual disturbances. However, its concurrent presentation with a rectal carcinoid tumor is rarely observed. This study reports the history, biochemical, colonoscopic and immunohistochemical results of a 48-year-old female with acromegaly and hyperprolactinemia. Despite the large size and invasive nature of the pituitary adenoma to adjacent anatomical structures, she did not complain of any neurologic symptoms such as visual disturbance or headache. Immunohistochemical staining of the surgical specimen from the pituitary adenoma revealed that the tumor cells were positive for growth hormone (GH, prolactin (PRL, and thyroid stimulating hormone (TSH. Staining for pituitary-specific transcription factor-1 (Pit-1 was shown to be strongly positive, which could have been possibly contributing to the plurihormonality of this adenoma. Colonoscopy found a rectal polyp that was identified to be a carcinoid tumor using immunohistochemical staining. A macroinvasive pituitary adenoma with concomitant rectal carcinoid tumor was secreting GH, PRL, and TSH, which were believed to be in association with over-expression of Pit-1. This is the first case report of double primary tumors comprising a plurihormonal pituitary macroadenoma and rectal carcinoid tumor.

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

    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.

  12. Polymorphonuclear neutrophil granulocyte chemotactic hyperresponsiveness in a case of canine acromegaly.

    Jensen, A L; Thomsen, M K; Aaes, H; Andreasen, M; Søndergaard, J

    1993-08-01

    Growth hormone (GH) has recently been shown to affect polymorphonuclear neutrophil granulocyte (PMN) function and to be secreted by mononuclear cells, indicating that the hormone may be active in an immunophysiologic network, acting as an endo- or paracrine priming agent. The purpose of the present study was to evaluate the chemotactic responsiveness of canine peripheral PMN in a dog with acromegaly, caused by spontaneous, progesterone-induced hypersecretion of GH and, secondary to this, a seven-fold increase in insulin-like growth factor I (IGF-I). The chemotactic responsiveness towards zymosan-activated serum (ZAS) and leukotriene B4 (LTB4) was evaluated at a time when the dog suffered from acromegaly and again 57 days after corrective surgery (ovariohysterectomy). The experiments showed that PMN from the patient exhibited enhanced chemotactic migration that appeared to be associated with the hypersomatotropic condition as judged from the reversibility of the phenomenon. The glucose intolerance and elevated serum alkaline phosphatase that were observed in the acromegalic dog were also shown to be reversible following surgery.

  13. Molecular Characterization of Growth Hormone-producing Tumors in the GC Rat Model of Acromegaly.

    Martín-Rodríguez, Juan F; Muñoz-Bravo, Jose L; Ibañez-Costa, Alejandro; Fernandez-Maza, Laura; Balcerzyk, Marcin; Leal-Campanario, Rocío; Luque, Raúl M; Castaño, Justo P; Venegas-Moreno, Eva; Soto-Moreno, Alfonso; Leal-Cerro, Alfonso; Cano, David A

    2015-11-09

    Acromegaly is a disorder resulting from excessive production of growth hormone (GH) and consequent increase of insulin-like growth factor 1 (IGF-I), most frequently caused by pituitary adenomas. Elevated GH and IGF-I levels results in wide range of somatic, cardiovascular, endocrine, metabolic, and gastrointestinal morbidities. Subcutaneous implantation of the GH-secreting GC cell line in rats leads to the formation of tumors. GC tumor-bearing rats develop characteristics that resemble human acromegaly including gigantism and visceromegaly. However, GC tumors remain poorly characterized at a molecular level. In the present work, we report a detailed histological and molecular characterization of GC tumors using immunohistochemistry, molecular biology and imaging techniques. GC tumors display histopathological and molecular features of human GH-producing tumors, including hormone production, cell architecture, senescence activation and alterations in cell cycle gene expression. Furthermore, GC tumors cells displayed sensitivity to somatostatin analogues, drugs that are currently used in the treatment of human GH-producing adenomas, thus supporting the GC tumor model as a translational tool to evaluate therapeutic agents. The information obtained would help to maximize the usefulness of the GC rat model for research and preclinical studies in GH-secreting tumors.

  14. Pituitary MRI characteristics in 297 acromegaly patients based on T2-weighted sequences.

    Potorac, Iulia; Petrossians, Patrick; Daly, Adrian F; Schillo, Franck; Ben Slama, Claude; Nagi, Sonia; Sahnoun, Mouna; Brue, Thierry; Girard, Nadine; Chanson, Philippe; Nasser, Ghaidaa; Caron, Philippe; Bonneville, Fabrice; Raverot, Gérald; Lapras, Véronique; Cotton, François; Delemer, Brigitte; Higel, Brigitte; Boulin, Anne; Gaillard, Stéphan; Luca, Florina; Goichot, Bernard; Dietemann, Jean-Louis; Beckers, Albert; Bonneville, Jean-François

    2015-04-01

    Responses of GH-secreting adenomas to multimodal management of acromegaly vary widely between patients. Understanding the behavioral patterns of GH-secreting adenomas by identifying factors predictive of their evolution is a research priority. The aim of this study was to clarify the relationship between the T2-weighted adenoma signal on diagnostic magnetic resonance imaging (MRI) in acromegaly and clinical and biological features at diagnosis. An international, multicenter, retrospective analysis was performed using a large population of 297 acromegalic patients recently diagnosed with available diagnostic MRI evaluations. The study was conducted at ten endocrine tertiary referral centers. Clinical and biochemical characteristics, and MRI signal findings were evaluated. T2-hypointense adenomas represented 52.9% of the series, were smaller than their T2-hyperintense and isointense counterparts (Pdiagnosis than women (P=0.067) and presented higher IGF1 values (P=0.01). Although in total, adenomas had a predominantly inferior extension in 45.8% of cases, in men this was more frequent (P<0.0001), whereas in women optic chiasm compression of macroadenomas occurred more often (P=0.0067). Most adenomas (45.1%) measured between 11 and 20 mm in maximal diameter and bigger adenomas were diagnosed at younger ages (P=0.0001). The T2-weighted signal differentiates GH-secreting adenomas into subgroups with particular behaviors. This raises the question of whether the T2-weighted signal could represent a factor in the classification of acromegalic patients in future studies.

  15. Spontaneous remission of acromegaly: apoplexy mimicking meningitis or meningitis as a cause of apoplexy?

    Villar-Taibo, Rocío; Ballesteros-Pomar, María D; Vidal-Casariego, Alfonso; Alvarez-San Martín, Rosa M; Kyriakos, Georgios; Cano-Rodríguez, Isidoro

    2014-02-01

    Pituitary apoplexy is a rare but potentially life-threatening clinical syndrome characterized by ischemic infarction or hemorrhage into a pituitary tumor. The diagnosis of pituitary tumor apoplexy is frequently complicated because of the nonspecific nature of its signs and symptoms, which can mimic different neurological processes, including meningitis. Several factors have been associated with apoplexy, such as dopamine agonists, radiotherapy, or head trauma, but meningitis is a rarely reported cause. We describe the case of a 51-year-old woman with acromegaly due to a pituitary macroadenoma. Before surgical treatment, she arrived at Emergency with fever, nausea, vomiting and meningismus. Symptoms and laboratory tests suggested bacterial meningitis, and antibiotic therapy was initiated, with quick improvement. A computerized tomography (CT) scan at admission did not reveal any change in pituitary adenoma, but a few weeks later, magnetic resonance imaging (MRI) showed data of pituitary apoplexy with complete disappearance of the adenoma. Currently, her acromegaly is cured, but she developed hypopituitarism and diabetes insipidus following apoplexy. We question whether she really experienced meningitis leading to apoplexy or whether apoplexy was misinterpreted as meningitis. In conclusion, the relationship between meningitis and pituitary apoplexy may be bidirectional. Apoplexy can mimic viral or bacterial meningitis, but meningitis might cause apoplexy, as well. This fact highlights the importance of differential diagnosis when evaluating patients with pituitary adenomas and acute neurological symptoms.

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

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

    2015-06-01

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

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

    Jain Kapil

    2008-01-01

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

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

    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.

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

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

    2010-01-01

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

  20. 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?

    Dal J; Skou N; Nielsen EH; Jørgensen JOL; Pedersen L

    2014-01-01

    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 ...

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

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

    2005-05-01

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

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

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

    2016-07-18

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

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

    Bonert Vivien S

    2010-05-01

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

  4. 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?

    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

  5. Stochastic Model to Find the Gallbladder Motility in Acromegaly Using Exponential Distribution

    P. Senthil Kumar

    2014-08-01

    Full Text Available The purpose of the study was octreotide therapy in acromegaly is associated with an increased prevalence of gall stones, which may be the result of inhibition of gall bladder motility. Gall stone prevalence in untreated acromegalic patients relative to the general population is unknown, however and the presence of gall stones and gall bladder motility in these patients and in acromegalic patients receiving octreotide was therefore examined. Gall bladder emptying in untreated acromegalic subjects is impaired. Octreotide further increases post prandial residual gall bladder volume and this may be a factor in the increased gall stone prevalence seen in these patients. The results of octreotide therapy in acromegalic and the normal controls were compared using the marginal distribution of a stretched Brownian motion ( as ∗ ; = 1 4 − 2 4

  6. Oxalate nephropathy induced by octreotide treatment for acromegaly: a case report

    Gariani Karim

    2012-07-01

    Full Text Available Abstract Introduction Oxalate nephropathy has various etiologies and remains a rare cause of renal failure. To the best of our knowledge, we report the first case of oxalate nephropathy following octreotide therapy. Case presentation We report the case of a 78-year-old Caucasian man taking chronic octreotide treatment for acromegaly who presented with acute oxalate nephropathy after antibiotic therapy. The diagnosis was confirmed by urinary analysis and a kidney biopsy. The recovery of renal function was favorable after hydration and withdrawal of octreotide therapy. Conclusions Oxalate nephropathy should be suspected in patients at risk who present with acute kidney injury after prolonged antibiotic treatment. This diagnosis should be distinguished from immuno-allergic interstitial nephritis and requires specific care. The evolution of this condition may be favorable if the pathology is identified correctly. Octreotide therapy should be considered a risk factor for enteric oxaluria.

  7. Long-term treatment of acromegaly with a long-acting analogue of somatostatin, octreotide.

    Page, M D; Millward, M E; Taylor, A; Preece, M; Hourihan, M; Hall, R; Scanlon, M F

    1990-02-01

    We have treated 16 acromegalic patients for up to 44 months with octreotide in varying doses. Growth hormone levels were suppressed in 14 patients with associated clinical improvement. IGF-1 levels were measured in 12 and fell into the normal range in 10. Prolactin was suppressed in six hyperprolactinaemic patients but was unaltered in normoprolactinaemic acromegalic patients. Post-prandial hyperglycaemia with impaired insulin secretion was noted in all patients, and one patient required oral hypoglycaemic agents. Octreotide did not affect thyroid function. CT scans from before and after six months of treatment demonstrated minimal tumour shrinkage in only two patients. Octreotide was well tolerated with no serious haematological or biochemical disturbance and no evidence of malabsorption. Two patients developed gallstones. Octreotide is effective in acromegaly. The development of gallstones is the only serious adverse event we have encountered.

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

    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.

  9. Acromegaly accompanied by Turner syndrome with 47,XXX/45,X/46,XX mosaicism.

    Yamazaki, Masanori; Sato, Ai; Nishio, Shin-ichi; Takeda, Teiji; Miyamoto, Takahide; Katai, Miyuki; Hashizume, Kiyoshi

    2009-01-01

    A 33-year-old woman was hospitalized for examination of edematous laryngopharynx. She was acromegalic. A pituitary adenoma with elevated serum levels of growth hormone (GH) and insulin-like growth factor-I (IGF-I) was detected, indicating acromegaly caused by GH-secreting pituitary adenoma. Multiple pigmented nevi were also noted without overt short stature and cubitus valgus. Chromosome analysis revealed that she had contracted Turner syndrome with 47,XXX/45,X/46,XX mosaicism. Transsphenoidal resection of the tumor decreased serum GH and IGF-I levels, but the edema was not improved. Both premature ovarian failure and hypertension appeared after surgery. This case may indicate the important relationships between GH/IGF-I and Turner syndrome.

  10. Childhood acromegaly due to X-linked acrogigantism: long term follow-up

    Gordon, Rebecca J.; Bell, Jennifer; Chung, Wendy K.; David, Raphael; Oberfield, Sharon E.; Wardlaw, Sharon L.

    2017-01-01

    Purpose Acromegaly in infancy is extremely rare. We describe a 32 year old woman who presented at 6 months of age with isolated macrocephaly, followed by accelerated linear growth. At 21 months of age, her head circumference was 55 cm (+5.5 SD), height was 97.6 cm (+4.4 SD) and weight was 20.6 kg (+6.2 SD). She had markedly elevated levels of growth hormone (GH) (135 ng/ml), IGF-1 (1540 ng/ml) and prolactin (370 ng/ml). A pituitary macroadenoma was surgically resected. Immunohistochemical staining was positive for GH. Post-operatively, she developed ACTH and TSH deficiency and diabetes insipidus. Methods Long term clinical follow-up and genetic testing with chromosomal microarray analysis. Results Despite GH deficiency, she grew well until 7 ½ years old, with subsequent decline in growth velocity, and received GH therapy for 5 years. Puberty was initiated with estrogen therapy. As an adult, she has no stigmata of acromegaly, with a height of 164.5 cm and non-acromegalic features. IGF-1 has remained in the low normal range. Prolactin has been mildly elevated. Serial MRIs have shown no evidence of tumor recurrence. She receives replacement therapy with hydrocortisone, levothyroxine and DDAVP. Chromosomal microarray analysis revealed that she has X-linked acrogigantism (X-LAG) due to a de novo duplication of Xq26.3 (516 kb). She recently became pregnant following ovarian stimulation and chorionic villus sampling revealed that she is carrying a male with the same duplication. Conclusion This report provides detailed long term clinical follow-up of a patient with X-LAG syndrome. PMID:27631333

  11. Leptin Receptor Gene Polymorphism may Affect Subclinical Atherosclerosis in Patients with Acromegaly

    Turgut, Sebahat; Topsakal, Senay; Ata, Melek Tunç; Herek, Duygu; Akın, Fulya; Özkan, Şeyma; Turgut, Günfer

    2016-01-01

    Background: Acromegaly is associated with increased morbidity and mortality related to cardiovascular diseases. Leptin (LEP) and Leptin Receptor (LEPR) gene polymorphisms can increase cardiovascular risks. The aim of this study was to investigate association between the frequencies of LEP and LEPR gene polymorphisms and subclinical atherosclerosis in acromegalic patients. Methods: Forty-four acromegalic patients and 30 controls were admitted to study. The polymorphisms were identified by using polymerase chain reaction from peripheral blood samples. The levels of systolic and diastolic blood pressure, BMI, fasting plasma glucose, fasting insulin, IGF-I, GH, IGFBP3, leptin, triglyceride, carotid Intima Media Thickness (cIMT) and HDL and LDL cholesterol concentrations were evaluated. Results: There was statistically significant difference between the LEPR genotypes of acromegalic patients (GG 11.4%, GA 52.3%, and AA 36.4%) and controls (GG 33.3%, GA 50%, and AA 16.7%) although their LEP genotype distribution was similar. In addition, the prevalence of the LEPR gene G and A alleles was significantly different between patients and controls. No significant difference was found among the G(-2548) A leptin genotypes of groups in terms of the clinical parameters. cIMT significantly increased homozygote LEPR GG genotype group compared to AA subjects in patients. But the other parameters were not different between LEPR genotypes groups of patients and controls. Conclusion: It can be said that the LEPR gene polymorphism may affect cIMT in patients. The reason is that LEPR GG genotype carriers may have more risk than other genotypes in the development of subclinical atherosclerosis in acromegaly. PMID:27563428

  12. Meta-analysis on the effects of octreotide on tumor mass in acromegaly.

    Andrea Giustina

    Full Text Available BACKGROUND: The long-acting somatostatin analogue octreotide is used either as an adjuvant or primary therapy to lower growth hormone (GH levels in patients with acromegaly and may also induce pituitary tumor shrinkage. OBJECTIVE: We performed a meta-analysis to accurately assess the effect of octreotide on pituitary tumor shrinkage. DATA SOURCES: A computerized Medline and Embase search was undertaken to identify potentially eligible studies. STUDY ELIGIBILITY CRITERIA: Eligibility criteria included treatment with octreotide, availability of numerical metrics on tumor shrinkage and clear definition of a clinically relevant reduction in tumor size. Primary endpoints included the proportion of patients with tumor shrinkage and mean percentage reduction in tumor volume. DATA EXTRACTION AND ANALYSIS: The electronic search identified 2202 articles. Of these, 41 studies fulfilling the eligibility criteria were selected for data extraction and analysis. In total, 1685 patients were included, ranging from 6 to 189 patients per trial. For the analysis of the effect of octreotide on pituitary tumor shrinkage a random effect model was used to account for differences in both effect size and sampling error. RESULTS: Octreotide was shown to induce tumor shrinkage in 53.0% [95% CI: 45.0%-61.0%] of treated patients. In patients treated with the LAR formulation of octreotide, this increased to 66.0%, [95% CI: 57.0%-74.0%. In the nine studies in which tumor shrinkage was quantified, the overall weighted mean percentage reduction in tumor size was 37.4% [95% CI: 22.4%-52.4%], rising to 50.6% [95% CI: 42.7%-58.4%] with octreotide LAR. LIMITATIONS: Most trials examined were open-label and had no control group. CONCLUSIONS: Octreotide LAR induces clinically relevant tumor shrinkage in more than half of patients with acromegaly.

  13. Analysis of GPR101 and AIP genes mutations in acromegaly: a multicentric study.

    Ferraù, Francesco; Romeo, P D; Puglisi, S; Ragonese, M; Torre, M L; Scaroni, C; Occhi, G; De Menis, E; Arnaldi, G; Trimarchi, F; Cannavò, S

    2016-12-01

    This multicentric study aimed to investigate the prevalence of the G protein-coupled receptor 101 (GPR101) p.E308D variant and aryl hydrocarbon receptor interacting protein (AIP) gene mutations in a representative cohort of Italian patients with acromegaly. 215 patients with GH-secreting pituitary adenomas, referred to 4 Italian referral centres for pituitary diseases, have been included. Three cases of gigantism were present. Five cases were classified as FIPA. All the patients have been screened for germline AIP gene mutations and GPR101 gene p.E308D variant. Heterozygous AIP gene variants have been found in 7 patients (3.2 %). Five patients carried an AIP mutation (2.3 %; 4 females): 3 patients harboured the p.R3O4Q mutation, one had the p.R304* mutation and the last one the IVS3+1G>A mutation. The prevalence of AIP mutations was 3.3 % and 2.8 % when considering only the patients diagnosed when they were <30 or <40-year old, respectively. Furthermore, 2.0 % of the patients with a pituitary macroadenoma and 4.2 % of patients resistant to somatostatin analogues treatment were found to harbour an AIP gene mutation. None of the patients was found to carry the GPR101 p.E308D variant. The prevalence of AIP gene mutations among our sporadic and familial acromegaly cases was similar to that one reported in previous studies, but lower when considering only the cases diagnosed before 40 years of age. The GPR101 p.E308D change is unlikely to have a role in somatotroph adenomas tumorigenesis, since none of our sporadic or familial patients tested positive for this variant.

  14. Metabolic glucose status and pituitary pathology portend therapeutic outcomes in acromegaly.

    Sonia Cheng

    Full Text Available INTRODUCTION: Acromegaly is frequently associated with impaired glucose tolerance and/or diabetes. To evaluate the relationship between glucose metabolism and acromegaly disease, we evaluated 269 consecutive patients from two referral centres. METHODS: Clinical presentation, pituitary tumor size and invasiveness, and pituitary pathology were captured in a dedicated database. RESULTS: 131 women and 138 men with a mean age of 53.8 years were included. Of these, 201 (74.7% presented with a macroadenoma and 18 (6.7% with a microadenoma. Radiographic invasion was present in 91 cases (33.8%. Mean tumor diameter was 1.86 cm (0.2-4.6. Pituitary histopathologic findings revealed pure GH-producing somatotroph adenomas (SA in 147 patients, prolactin-production by mixed lactotroph (LA and SA or mammosomatotroph adenoma (MSA in 46 [22.4%], acidophil stem cell adenoma in 6 [2.9%], and other diagnoses in 6 [2.9%]. Medical treatment included octreotide in 96 [36.9%] and in combination with pegvisomant or dopamine agonists in 63 [24.2%]. Nearly 80% of patients achieved IGF-1 normalization. Importantly, patients with pure somatotroph adenomas were significantly more likely to present with abnormal glucose metabolism [48.7%] than those with mixed adenomas [9.7%] [p<0.001] independent of GH/IGF-1 levels or tumor invasiveness. Abnormal glucose metabolism and pituitary pathology also remained linked following IGF-1 normalization. Moreover patients with pure SA and abnormal glucose metabolism were significantly (p<0.001 less likely to achieve disease remission despite the same therapeutic strategies. Conversely, patients with mixed adenomas were more likely (OR: 2.766 (95% CI: 1.490-5.136 to achieve disease remission. CONCLUSIONS: Patients with pure somatotroph adenomas are more likely than those with mixed adenomas to exhibit abnormal glucose metabolism.

  15. Pituitary tumor disappearance in a patient with newly diagnosed acromegaly primarily treated with octreotide LAR.

    Resmini, E; Murialdo, G; Giusti, M; Boschetti, M; Minuto, F; Ferone, D

    2005-02-01

    We describe the case of an acromegalic patient primarily treated with octreotide LAR in whom the pituitary tumor disappeared after 18 months of treatment. A 68-yr-old woman, with clinical suspicion of acromegaly, was admitted to our Unit with the ultrasonographical evidence of cardiac hypertrophy, arrhythmias, right branch block and interatrial septum aneurism. She referred hands and feet enlargement since the age of 30 and facial disfigurements since the age of 50. At the age of 45 she underwent surgery for carpal tunnel syndrome and at the age of 61 an euthyroid nodular goiter was diagnosed. Hormonal evaluation showed elevated circulating GH levels (25+/-3.2 ng/ml), not suppressible after oral glucose load, and elevated IGF-I levels (646 ng/ml), whereas the remaining pituitary function was normal. Visual perimetry was normal, whereas magnetic resonance imaging (MRI) showed an intrasellar pituitary adenoma with maximal diameter of 9 mm. In order to improve cardiovascular function before surgery, the patient started octreotide LAR 20 mg every 4 weeks for 3 months. Then based on IGF-I values, the dose was adjusted to 30 mg. After 6 months a second MRI showed significant tumor reduction (>50% of baseline maximal diameter), GH and IGF-I were within the normal range and the patient continued the treatment. After one-year therapy, an improvement of cardiac alterations was recorded and the patient was referred to the neurosurgeon. However, she refused the operation. At 18-month follow-up, MRI showed the complete disappearance of direct and indirect signs of pituitary adenoma. To our knowledge, this is the first case of complete radiological remission of pituitary tumor during octreotide LAR treatment in acromegaly.

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

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

    2010-01-01

    of acromegaly is initiated. This was a three months prospective study investigating short-term cardiac effects of treatment in acromegalic patients. Cardiac function was evaluated by the gold standard method cardiac magnetic resonance imaging (CMRI) and circulating levels of B-type natriuretic peptides (BNP......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...... and NT-proBNP). CMRI was performed at baseline and after 3 months of treatment. Levels of IGF-I, BNP and NT-proBNP were measured after 0, 1, 2 and 3 months. Eight patients (5 males and 3 females, mean age 53 ± 12 years (range 30-70)) and 8 matched healthy control subjects were included. Median IGF-I Z...

  17. Refractory DKA as first presentation of acromegaly and a potential role for continuous venovenous hemofiltration in its successful management.

    Mewawalla, Prerna; Jaiswal, Gayatri; Moustakakis, Michael; Sankaranarayanan, Niranjan; Dasanu, Constantin A

    2011-08-01

    Diabetic ketoacidosis is rarely encountered in acromegaly. We present a unique patient with refractory diabetic ketoacidosis (DKA) as a first presentation of acromegaly. In addition to an insulin drip and intravenous fluids, our patient was managed with octreotide therapy. As he developed acute renal failure in the context of renal hypoperfusion, continuous venovenous hemofiltration (CVVH) was instituted. After only three days of therapy, the growth hormone (GH) level dropped circa fourfold and insulin growth factor 1 (IGF-1) level dropped ninefold. We postulate a hypothetical role of CVVH in removal of plasma GH and IGF-1, similar to the clearance of other medium size molecules such as brain natriuretic peptide and procalcitonin. If this is confirmed in future studies, CVVH may have therapeutic implications for the above category of patients.

  18. Partial purification and characterization of a peptide with growth hormone-releasing activity from extrapituitary tumors in patients with acromegaly.

    Frohman, L A; Szabo, M; Berelowitz, M.; Stachura, M E

    1980-01-01

    Growth hormone (GH)-releasing activity has been detected in extracts of carcinoid and pancreatic islet tumors from three patients with GH-secreting pituitary tumors and acromegaly. Bioactivity was demonstrated in 2 N acetic acid extracts of the tumors using dispersed rat adenohypophyseal cells in primary monolayer culture and a rat anterior pituitary perifusion system. The GH-releasing effect was dose responsive and the greatest activity was present in the pancreatic islet tumor. Small amount...

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

    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.

  20. Pharmacokinetics, pharmacodynamics, and safety of pasireotide LAR in patients with acromegaly: a randomized, multicenter, open-label, phase I study.

    Petersenn, Stephan; Bollerslev, Jens; Arafat, Ayman M; Schopohl, Jochen; Serri, Omar; Katznelson, Laurence; Lasher, Janet; Hughes, Gareth; Hu, Ke; Shen, George; Reséndiz, Karina Hermosillo; Giannone, Vanessa; Beckers, Albert

    2014-11-01

    Pasireotide (SOM230), a multireceptor-targeted somatostatin analogue, has exhibited favorable safety/tolerability in several clinical studies. A long-acting-release (LAR) formulation of pasireotide may offer advantages over the subcutaneous formulation. This randomized, open-label, Phase I study evaluated the safety, PK, and PD of pasireotide LAR 20, 40, or 60 mg/month in patients with acromegaly. Safety assessments and blood samples for PK and PD were taken at designated time points. Thirty-five patients were randomized and completed the study. Steady-state pasireotide concentrations were achieved following three monthly injections. Trough pasireotide concentrations (ng/mL) 28 days after each injection were: 2.48, 4.16, and 3.10 (20 mg group); 6.42, 6.62, and 7.12 (40 mg group); and 9.51, 11.7, and 13.0 (60 mg group). At study end, 51% and 57% of patients achieved GH levels ≤2.5 μg/L and IGF-1 levels below ULN, respectively. Compared with baseline, fasting blood glucose and HbA1c levels increased, whereas fasting blood insulin levels decreased. Acromegaly symptoms were generally improved. Adverse events were mostly gastrointestinal and mild/moderate. Pasireotide LAR was generally well tolerated. Steady-state PK was achieved after three monthly doses; exposures were approximately dose proportional. Control of GH, IGF-1, and symptoms improved, suggesting that pasireotide LAR may be an effective treatment for acromegaly.

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

    Joanna Matrozova

    2014-02-01

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

  2. Case Report: A case report of acromegaly associated with primary aldosteronism [v2; ref status: indexed, http://f1000r.es/3ke

    Joanna Matrozova

    2014-06-01

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

  3. Gender differences in serum GH and IGF-I levels and the GH response to dynamic tests in patients with acromegaly.

    Tanaka, Satoshi; Fukuda, Izumi; Hizuka, Naomi; Takano, Kazue

    2010-01-01

    Gender affects the GH secretory pattern both in normal subjects and in patients with acromegaly by an uncertain mechanism. Here, we report the influence of gender on the relationship between serum GH and IGF-I levels and the GH response to dynamic tests in patients with acromegaly. Seventy-four patients with untreated acromegaly (M/F 27/47, age range 22-86 yr.) were studied. The serum GH levels did not differ between male and female (6.1 vs. 8.7 ng/ml; p=0.26), while serum IGF-I levels, IGF-I SDS and the IGF-I/GH ratio were lower in female than those in male (679 vs. 769 ng/ml; p50 yr, serum IGF-I levels and IGF-I/GH ratios were lower in female than those in male in patients 50 yrs (684 vs. 680 ng/ml; p=0.39 and 98.7 vs. 118.4; p=0.40). The GH responses to OGTT, TRH, octreotide, and bromocriptine tests were similar in male and female. In conclusion, IGF-I/GH ratio was significantly lower in female than that in male particularly in younger patients with acromegaly. These data suggest that gender, presumably sex steroids in female, may partially modulate the relationship between circulating IGF-I and GH levels in patients with acromegaly.

  4. Patología cardiovascular en la acromegalia Cardiovascular Disease in Acromegaly

    L Fiszlejder

    2012-09-01

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

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

    Andreassen, Mikkel; Frystyk, Jan; Miller, K.K.;

    2010-01-01

    We describe a 34-year-old female treated with IFN-beta for 8 years with a biochemical profile suggestive of acromegaly. The patient presented with elevated serum insulin-like growth factor-I (IGF-I) and insufficient suppression of growth hormone (GH) during oral glucose tolerance test (OGTT......). There were no clinical features of acromegaly. A 5-day profile showed higher GH levels on the 3 days following IFN-beta injections. Total and bioactive IGF-I were also elevated but did not fluctuate. Four weeks off IFN-beta normalized suppression of GH during OGTT but did not reduce serum IGF-I or bioactive...... IGF-I. In conclusion, IFN-beta treatment mimicked acromegaly biochemically. The changes were partially reversible...

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

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

    2001-01-01

    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...

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

    Skjaerbaek, Christian; Frystyk, Jan; Kaal, Andreas;

    2000-01-01

    to the nocturnal increase in IGF binding protein-1. In this study we have investigated the circadian variation in circulating free IGF-I and IGF-II in patients with acromegaly and patients with adult onset growth hormone deficiency. PATIENTS: Seven acromegalic patients were studied with and without treatment...... no significant circadian variations in free IGF-I or free IGF-II in either of the two occasions. In contrast, there was a significant circadian variation of total IGF-I after adjustment for changes in plasma volume in both treated and untreated acromegaly and GH deficiency in all cases with a peak between 0300 h...

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

    Losa, Marco; Bollerslev, Jens

    2016-06-01

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

  9. Cardiovascular events in acromegaly: distinct role of Agatston and Framingham score in the 5-year prediction.

    Ragonese, Marta; Alibrandi, Angela; Di Bella, Gianluca; Salamone, Ignazio; Puglisi, Soraya; Cotta, Oana Ruxandra; Torre, Maria Luisa; Ferrau, Francesco; Ruggeri, Rosaria Maddalena; Trimarchi, Francesco; Cannavo, Salvatore

    2014-09-01

    Prediction of ischemic cardiovascular events (ICE) in acromegalic patients stratified accordingly with Framingham (FS) and Agatston score (AS). 32 patients with active (group A (0)) and 20 with controlled (group B (0)) acromegaly have been enrolled. During the 5-year follow-up, 19 out of 32 patients in group A (0) reached disease control. At entry, FS and AS, by an eight-slice MDCT scanner, were calculated in all patients. ICE were diagnosed by autopsy, if lethal, and by electrocardiography and/or echocardiography, if non-lethal. Overall, 9.6 % of patients died for lethal ICE. AS >400, but not high FS at entry, was associated with increased risk of lethal ICE. Lethal ICE had occurred in two patients of group A (0) and three of group B (0) (p NS), while a non-lethal ICE had occurred in two cases of the former and in other two of the latter group (p NS). Either FS or AS was correlated with the risk for ICE overall (p 400 is associated with increased risk of lethal ICE, while high FS is associated with reduced life expectancy, regardless of disease control.

  10. Carcinoid syndrome, acromegaly, and hypoglycemia due to an insulin-secreting neuroendocrine tumor of the liver.

    Furrer, J; Hättenschwiler, A; Komminoth, P; Pfammatter, T; Wiesli, P

    2001-05-01

    We report a patient with a hepatic neuroendocrine tumor showing an extraordinary change of the tumor's humoral manifestations from a clinically documented extrapituitary acromegaly and a typical carcinoid syndrome toward a hyperinsulinemic hypoglycemia syndrome. At the primary manifestation of the tumor, an increased serum level of insulin-like growth factor I due to overproduction of GHRH and an increased urinary excretion of 5-hydroxyindoleacetic acid were found. The clinical manifestation of the GHRH excess was an arthralgia, which resolved completely after operative tumor debulking and normalization of insulin-like growth factor I and GHRH serum levels. The secretion of serotonin from the tumor resulted in a typical carcinoid syndrome including right-sided valvular heart disease. On the later course of the disease, the humoral manifestations of the tumor were supplemented by the secretion of insulin, leading to recurrent severe hyperinsulinemic hypoglycemia. The hepatic origin of hyperinsulinism was demonstrated by selective arterial calcium stimulation. Moreover, tumor cells revealed insulin and C-peptide immunoreactivity in the immunohistochemical analysis. The patient died 8 yr after the initial diagnosis of the tumor, and a carefully performed autopsy procedure confirmed the absence of any extrahepatic tumor manifestation.

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

    Francisco Pita-Gutierrez

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

  12. Predictive value of T2 relative signal intensity for response to somatostatin analogs in newly diagnosed acromegaly

    Shen, Ming; Zhang, Qilin [Fudan University, Department of Neurosurgery, Huashan Hospital, Shanghai Medical College, Shanghai (China); Shanghai Pituitary Tumor Center, Shanghai (China); Liu, Wenjuan; Li, Yiming; Zhang, Zhaoyun; Ye, Hongying; He, Min; Lu, Bin; Yang, Yeping [Shanghai Pituitary Tumor Center, Shanghai (China); Fudan University, Department of Endocrinology and Metabolism, Huashan Hospital, Shanghai Medical College, Shanghai (China); Wang, Meng [Fudan University, Department of Endocrinology and Metabolism, Huashan Hospital, Shanghai Medical College, Shanghai (China); Soochow University, Division of Endocrinology, the Second Affiliated Hospital, Suzhou (China); Zhu, Jingjing [Shanghai Pituitary Tumor Center, Shanghai (China); Fudan University, Department of Neuropathology, Huashan Hospital, Shanghai Medical College, Shanghai (China); Ma, Zengyi; He, Wenqiang; Li, Shiqi; Shou, Xuefei; Qiao, Nidan; Ye, Zhao; Zhang, Yichao; Zhao, Yao; Wang, Yongfei [Fudan University, Department of Neurosurgery, Huashan Hospital, Shanghai Medical College, Shanghai (China); Shanghai Pituitary Tumor Center, Shanghai (China); Yao, Zhenwei [Shanghai Pituitary Tumor Center, Shanghai (China); Fudan University, Department of Radiology, Huashan Hospital, Shanghai Medical College, Shanghai (China); Lu, Yun [Fudan University, Department of Nuclear Medicine, Huashan Hospital, Shanghai Medical College, Shanghai (China)

    2016-11-15

    The difficulty of predicting the efficacy of somatostatin analogs (SSA) is not fully resolved. Here, we quantitatively evaluated the predictive value of relative signal intensity (rSI) on T1- and T2-weighted magnetic resonance imaging (MRI) for the short-term efficacy (3 months) of SSA therapy in patients with active acromegaly and assessed the correlation between MRI rSI and expression of somatostatin receptors (SSTR). This was a retrospective review of prospectively recorded data. Ninety-two newly diagnosed patients (37 males and 55 females) with active acromegaly were recruited. All patients were treated with pre-surgical SSA, followed by reassessment and transspenoidal surgery. rSI values were generated by calculating the ratio of SI in the tumor to the SI of normal frontal white matter. The Youden indices were calculated to determine the optimal cutoff of rSI to determine the efficacy of SSA. The correlation between rSI and expression of SSTR2/5 was analyzed by the Spearman rank correlation coefficient. T2 rSI was strongly correlated with biochemical sensitivity to SSA. The cutoff value of T2 rSI to distinguish biochemical sensitivity was 1.205, with a positive predictive value (PPV) of 81.5 % and a negative predictive value (NPV) of 77.3 %. No correlation was found between MRI and tumor size sensitivity. Moreover, T2 rSI was negatively correlated with the expression of SSTR5. T2 rSI correlates with the expression of SSTR5 and quantitatively predicts the biochemical efficacy of SSA in acromegaly. (orig.)

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

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

    2010-01-01

    We describe a 34-year-old female treated with IFN-ß for 8 years with a biochemical profile suggestive of acromegaly. The patient presented with elevated serum insulin-like growth factor-I (IGF-I) and insufficient suppression of growth hormone (GH) during oral glucose tolerance test (OGTT). There ......We describe a 34-year-old female treated with IFN-ß for 8 years with a biochemical profile suggestive of acromegaly. The patient presented with elevated serum insulin-like growth factor-I (IGF-I) and insufficient suppression of growth hormone (GH) during oral glucose tolerance test (OGTT......). There were no clinical features of acromegaly. A 5-day profile showed higher GH levels on the 3 days following IFN-ß injections. Total and bioactive IGF-I were also elevated but did not fluctuate. Four weeks off IFN-ß normalized suppression of GH during OGTT but did not reduce serum IGF-I or bioactive IGF......-I. In conclusion, IFN-ß treatment mimicked acromegaly biochemically. The changes were partially reversible....

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

    W.W. de Herder (Wouter)

    2009-01-01

    textabstractIn 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

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

    Andries, Magdalene; Glintborg, Dorte; Kvistborg, Annette

    2007-01-01

    of acromegaly in a randomized study design. Material and methods Twelve acromegalic patients were included and 10 patients completed treatment with LAN or OCT for 6 months and were then switched to the opposite treatment modality for 6 months without a washout period in a randomized crossover design. GH and IGF...

  16. Quality of life is impaired in association with the need for prolonged postoperative therapy by somatostatin analogs in patients with acromegaly

    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.

    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 T

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

    Pinaki Dutta

    2015-01-01

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

  18. Persistent self-consciousness about facial appearance, measured with the Derriford appearance scale 59, in patients after long-term biochemical remission of acromegaly

    Roerink, S.H.P.P.; Wagenmakers, M.A.E.M.; Wessels, J.F.; Sterenborg, R.B.; Smit, J.W.A.; Hermus, A.R.M.M.; Netea-Maier, R.T.

    2015-01-01

    CONTEXT: Acromegaly is associated with impaired quality of life (QoL) and causes anatomical disproportions, which may contribute to the decreased QoL after successful treatment. The Derriford appearance scale 59 (DAS59) is a questionnaire measuring psychological distress and disruptions to everyday

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

    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

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

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

    2016-01-01

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

  1. Risk of colorectal neoplasm in patients with acromegaly: A meta-analysis

    Theodoros Rokkast; Dimitrios Pistiolas; Panos Sechopoulos; Georgios Margantinis; Georgios Koukoulis

    2008-01-01

    AIM: To examine the risk of colorectal neoplasm in acromegalic patients by meta-analyzing all relevant controlled studies.METHODS: Extensive English language medical literature searches for human studies, up to December 2007, were performed using suitable keywords. Pooled estimates [odds ratio (OR) with 95% confidence intervals (CI)] were obtained using either the fixed or random-effects model as appropriate. Heterogeneity between studies was evaluated with the Cochran Q test whereas the likelihood of publication bias was assessed by constructing funnel plots. Their symmetry was estimated by the adjusted rank correlation test.RESULTS: For hyperplastic polyps the pooled ORs with 95% CI were 3.557(2.587-4.891) by fixed effects model and 3.703(2.565-5.347) by random effects model. The Z test values for overall effect were 7.81 and 6.984, respectively (P<0.0001). For colon adenomas the pooled ORs with 95% CI were 2.486(1.908-3.238) (fixed effects model) and 2.537(1.914-3.364) (random effects model). The Z test values were 6.747 and 6.472, respectively (P<0.0001). For colon cancer the pooled OR with 95% CI was identical for both fixed and random effects model (OR, 4.351; 95% CI, 1.533-12.354; Z=2.762, P=0.006]. There was no significant heterogeneity and no publication bias in all the above meta-analyses.CONCLUSION: Acromegaly is associated with an increased risk of colorectal neoplasm.

  2. Bromocriptine therapy in acromegaly. A long-term review of 35 cases.

    Sachdev, Y; Gopal, K; Garg, V K

    1981-04-01

    Bromocriptine (CB-154, Parlodel, Sandoz) was given to 35 acromegalic patients for a period of 6-36 months. Basal and post-therapy endocrine functions including estimation of serum growth hormone (GH) profile; and GH kinetics during oral glucose tolerance test, augmented insulin tolerance test and thyrotrophin releasing hormone test were determined. The pituitary tumour size was delineated by a pneumoencephalogram. The mean GH levels ranged from 14 micrograms/l to 316 micrograms/l. Bromocriptine suppressed GH values to 5 micrograms/l or less in 16 patients and less than 10 micrograms/l in a further 6 patients. In 33 patients GH values fell to 50% of the basal value or less. There was no significant GH reduction in 2 'nonresponders'. Bromocriptine did not block the stress-induced GH secretion. It did not disturb pituitary functions other than prolactin which was suppressed much earlier and was maintained with smaller doses. GH suppression on the other hand was shortlived and rebounded when the drug was omitted. It had no adverse effect on tumour size in 2 patients having suprasellar extension of the tumour. Bromocriptine improved carbohydrate tolerance and sexual function although it did not affect insulin and gonadotrophin values. It seems reasonable to offer a trial of bromocriptine in all patients with acromegaly where therapy is deemed necessary as it is well tolerated, has insignificant side effects and no adverse drug interactions. Its high cost and prolonged course are obvious disadvantages. Caution should be exercised in cases with suprasellar extension and visual field involvement.

  3. Advances in drug treatment of acromegaly%肢端肥大症的药物治疗进展

    周良; 张义

    2015-01-01

    肢端肥大症是一种隐袭起病、缓慢进展的内分泌疾病。肢端肥大症主要由生长激素垂体瘤引起,这种垂体瘤长期、过度分泌生长激素导致循环胰岛素样生长因子-1(insulin-like growth factor-1,IGF-1)升高,从而导致靶器官受损、增加患者死亡率并且降低生活质量。本文检索5年来的相关文献,回顾肢端肥大症的药物治疗进展。%Acromegaly is mostly caused by a pituitary adenoma secreting growth hormone ( GH ) , which leads to a wide range of endocrine morbidities, with an insidious onset and slow progression.A long-term overproduction of GH results in elevated insulin-like growth factor-1 ( IGF-1 ) level, which in turn, would cause target organ damage and an increased risk for mortality and decreased quality of life.Herein, the literature regarding the progress of drug therapy for acromegaly in recent five years is reviewed.

  4. Association between the Growth Hormone Receptor Exon 3 Polymorphism and Metabolic Factors in Korean Patients with Acromegaly

    Hye Yoon Park

    2015-09-01

    Full Text Available BackgroundThis study investigated the association between the frequency of growth hormone receptor (GHR exon 3 polymorphism (exon 3 deletion; d3-GHR and metabolic factors in patients with acromegaly in Korea.MethodsDNA was extracted from the peripheral blood of 30 unrelated patients with acromegaly. GHR genotypes were evaluated by polymerase chain reaction and correlated with demographic data and laboratory parameters.ResultsNo patient had the d3/d3 genotype, while four (13.3% had the d3/fl genotype, and 26 (86.7% had the fl/fl genotype. Body mass index (BMI in patients with the d3/fl genotype was significantly higher than in those with the fl/fl genotype (P=0.001. Age, gender, blood pressure, insulin-like growth factor-1, growth hormone, fasting plasma glucose, triglycerides, high density lipoprotein cholesterol, and low density lipoprotein cholesterol levels showed no significant differences between the two genotypes.ConclusionThe d3-GHR polymorphism may be associated with high BMI but not with other demographic characteristics or laboratory parameters.

  5. Low insulin resistance after surgery predicts poor GH suppression one year after complete resection for acromegaly: a retrospective study.

    Edo, Naoki; Morita, Koji; Suzuki, Hisanori; Takeshita, Akira; Miyakawa, Megumi; Fukuhara, Noriaki; Nishioka, Hiroshi; Yamada, Shozo; Takeuchi, Yasuhiro

    2016-05-31

    Remission of acromegaly is defined as a nadir in GH GH nadir (GH nadir of 0.4-1.0 ng/mL one year after complete resection of GH-secreting pituitary adenoma (GHoma). We included 110 cases of acromegaly with complete adenoma resection, no preoperative treatment, preoperative glycosylated hemoglobin GH nadir GH nadir GH (GH nadir >0.4 ng/mL). Clinical parameters, including measures of insulin secretion and resistance, were compared between groups. The high GH group included 10 patients (9.1%) and had a lesser level of insulin resistance immediately following surgery and at the first postoperative year evaluation. On single regression analysis, insulin resistance immediately following surgery was predictive of and correlated with the GH nadir at the first postoperative year evaluation. The GH nadir at the first postoperative year evaluation may be insufficient in patients with normalized IGF-1 with low insulin resistance immediately following complete resection of GHoma. Careful evaluation is needed to assess remission in such patients.

  6. Prevalence of lung structure abnormalities in patients with acromegaly and their relationship with gas exchange: cross-sectional analytical study with a control group

    Marcelo Palmeira Rodrigues

    Full Text Available CONTEXT AND OBJECTIVE: Different functional respiratory alterations have been described in acromegaly, but their relationship with pulmonary tissue abnormalities is unknown. The objective of this study was to observe possible changes in lung structure and explain their relationship with gas exchange abnormalities. DESIGN AND SETTING: Cross-sectional analytical study with a control group, conducted at a university hospital. METHODS: The study included 36 patients with acromegaly and 24 controls who were all assessed through high-resolution computed tomography of the thorax (CT. Arterial blood gas, effort oximetry and serum growth hormone (GH and insulin-like growth factor I (IGF-1 were also assessed in the patients with acromegaly. RESULTS: The abnormalities found in the CT scan were not statistically different between the acromegaly and control groups: mild cylindrical bronchiectasis (P = 0.59, linear opacity (P = 0.29, nodular opacity (P = 0.28, increased attenuation (frosted glass; P = 0.48 and decreased attenuation (emphysema; P = 0.32. Radiographic abnormalities were not associated with serum GH and IGF-1. Hypoxemia was present in seven patients; however, in six of them, the hypoxemia could be explained by underlying clinical conditions other than acromegaly: chronic obstructive pulmonary disease in two, obesity in two, bronchial infection in one and asthma in one. CONCLUSION: No changes in lung structure were detected through thorax tomography in comparison with the control subjects. The functional respiratory alterations found were largely explained by alternative diagnoses or had subclinical manifestations, without any plausible relationship with lung structural factors.

  7. 肢端肥大症最新诊疗指南%Guidelines for acromegaly management: an update

    朱慧娟; 曾正陪

    2009-01-01

    @@ 肢端肥大症共识工作组(Acromegaly Consensus Group,ACG)自2000年以来,在肢端肥大症治疗的各个方面公布了若干共识文件,2002年发表了全面的肢端肥大症诊疗指南,2007年11月再次召集会议讨论更新指南,在该组的第六次会议上,由垂体学会和欧洲神经内分泌协会资助的68名神经外科和内分泌专家达成共识并根据2007年的进展制定了新的肢端肥大症治疗指南.

  8. Criteria for cure in acromegaly: report of a case apparently cured in which persisting tumor was found at autopsy.

    Wrightson, P; Holdaway, I M; Synek, B J

    1984-06-01

    A 49-year-old man with acromegaly underwent the apparently complete removal of a pituitary microadenoma by the transsphenoidal route. There was complete remission of the biochemical abnormalities for 2 1/2 years, with basal plasma growth hormone (GH) levels of less than 1 ng/ml and normal somatomedin levels. The patient then died after the rupture of a cerebral aneurysm. Serial sections of the pituitary fossa showed persistent tumor with a volume of about 6% of the remaining normal gland. It is concluded that current endocrine tests are limited in their ability to detect residual GH-secreting tumor. Their sensitivity is likely to depend on the mass of tumor and on the GH secretion rate. Predictions of long term cure based on these tests are only tentative.

  9. The Coexistence of an Intrasellar Adenoma, Lymphocytic Hypophysitis, and Primary Pituitary Lymphoma in a Patient with Acromegaly

    Jose Hernan Martinez

    2011-01-01

    Full Text Available The concomitant presence of three histopathologically different entities in the pituitary gland is a rare occurrence. Most publications identify at least two distinct pathologies, mainly, a pituitary adenoma coexisting with a second intrasellar lesion. We present a case of a 71-year-old female referred for evaluation and treatment of acromegaly. Questioning revealed she was experiencing facial palsy, visual disturbances, and syncopal spells for several weeks. When laboratory evaluation showed elevated somatomedin (IGF-I levels and an oral glucose tolerance test failed to demonstrate any suppression of her growth hormone (GH values, an MRI of the pituitary revealed a sellar mass. A presumptive diagnosis of pituitary adenoma was established. The patient underwent transsphenoidal resection of the sellar mass, which proved to be a large B-cell lymphoma (Stage I-E associated with areas of adenoma and lymphocytic hypophysitis.

  10. Effects of theophylline infusion on the growth hormone (GH) and prolactin response to GH-releasing hormone administration in acromegaly.

    Losa, M; Alba-Lopez, J; Schopohl, J; Sobiesczcyk, S; Chiodini, P G; Müller, O A; von Werder, K

    1988-10-01

    Since theophylline has been shown to blunt the GH response to growth hormone-releasing hormone (GHRH) in normal subjects, we investigated whether the same effect of theophylline administration could be reproduced in patients with active acromegaly. Ten acromegalic patients received on two different days 100 micrograms GHRH iv alone and the same GHRH dose during a constant infusion of theophylline (3.56 mg/min), beginning 2 h before GHRH administration. In the whole group theophylline did not affect basal GH secretion significantly (from a mean of 44.6 +/- 14.4 at 0 min to 41.8 +/- 13.5 ng/ml at 120 min). However, the amount of GH released after GHRH stimulation was lower when theophylline was concomitantly infused (7525 +/- 3709 ng min/ml vs. 12038 +/- 6337 ng min/ml; p less than 0.05). The inhibitory effect of theophylline was not homogeneous, since either marked or minimal reductions of the GHRH-stimulated GH secretion occurred. Serum PRL levels increased after GHRH administration in 6 patients and theophylline infusion had no influence upon this response. Peak GHRH levels were not different in both studies (14.9 +/- 1.7 and 17.1 +/- 4.0 ng/ml, respectively). Free fatty acid levels rose progressively during theophylline administration (from 0.66 +/- 0.10 at 0 min to 1.04 +/- 0.10 mEq/l at 240 min) and were significantly higher than after GHRH stimulation alone from 180 min up to the end of the test. Our results demonstrate that in active acromegaly theophylline blunts the GH response to GHRH, though this effect is not uniformly seen in all patients.(ABSTRACT TRUNCATED AT 250 WORDS)

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

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

    2015-02-01

    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 Health Care could influence the outcome of the disease. Clinical data from 112 consecutive patients were collected and home addresses were plotted in the GIS software for spatial analysis. The buffer spatial distribution of patients living in Brasilia showed that 38.1% lived from 0.33 to 8.66 km, 17.7% from 8.67 to 18.06 km, 22.2% from 18.07 to 25.67 km and 22% from 25.68 to 36.70 km distant to the Reference Medical Center (RMC), and no unexpected clusters were identified. Migration of 26 patients from 11 others cities in different regions of the country was observed. Most of patients (64%) with adenomas bigger than 25 mm lived more than 20 km away from RMC, but no significant correlation between the distance from patient's home to the RMC and tumor diameter (r = 0.45 p = 0.20) nor for delay in diagnosis (r = 0.43 p = 0.30) was found. The geographical distribution of diagnosed cases did not impact in the latency of diagnosis or tumor size but the recognition of significant migration denotes that improvements in the medical assistance network are needed.

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

    Parkinson, C; Kassem, M; Heickendorff, Lene

    2003-01-01

    . Pegvisomant-induced normalization of serum IGF-I results in a decrease in markers of bone and soft tissue turnover to levels observed in age-matched controls, and these changes are accompanied by an increase in PTH and a decrease in 1,25-(OH)(2) vit D. These data provide further evidence of the effectiveness...... cross-linked N-telopeptide/creatinine ratio, and urinary calcium (24 h collection) were measured (single-batch analysis) at study entry and after IGF-I normalization, along with sera from 32 age- and sex-matched controls. Compared with controls, PIIINP, OC, and CTx were significantly elevated......Active acromegaly is associated with increased biochemical markers of bone turnover. Pegvisomant is a GH receptor antagonist that normalizes serum IGF-I in 97% of patients with active acromegaly. We evaluated the effects of pegvisomant-induced serum IGF-I normalization on biochemical markers...

  13. Failure of growth hormone-suppressing agents to affect TSH-releasing hormone- and LH-releasing hormone-induced growth hormone release in acromegaly.

    Nakagawa, K; Obara, T

    1977-01-01

    In patients with acromegaly whose basal plasma GH levels were suppressed with 9 mg/day of dexamethasone for 2 days, TRH-(6 cases) and LHRH-(1 case) induced GH release were unaffected when the responses were compared to the basal levels. Phentolamine infusion, 70 mg in 150 min, or hyperglycemia induced by iv infusion of 700 ml of 50% glucose solution also did not suppress TRH-induced GH release in 2 acromegalic patients whose basal GH levels were lowered with these agents alone. These results seem to indicate that dexamethasone does not affect TRH- or LHRH-induced GH release per se, but affects the basal state which determines the absolute level of response. They also support the concept that TRH and LHRH act directly on pituitary tumor cells to release GH in acromegaly.

  14. Prevalence of dermatologic disorders in 15 patients with acromegaly Prevalência de alterações dermatológicas em 15 pacientes acromegálicos

    Mariângela Resende

    2012-02-01

    Full Text Available Acromegaly is a chronic, insidious and rare disease, caused by hyper secretion of the growth hormone, which metabolic and trophic effects commonly result in cutaneous manifestations, sometimes preceding other clinical symptoms. The authors have assessed 15 patients with acromegaly and found dermatologic lesions in all of them, mainly skin thickening, acrochordons, epidermoid cysts, pseudoacanthosis nigricans, seborrheic keratosis, melanocytic naevi and lentiginous spots.Acromegalia é doença crônica rara, insidiosa, decorrente da hipersecreção de hormônio do crescimento, cujos efeitos tróficos e metabólicos frequentemente incorrem em manifestações cutâneas, que podem ser precoces. Os autores avaliaram 15 pacientes portadores de acromegalia e evidenciaram alterações dermatológicas em todos, principalmente espessamento da pele, acrocórdons, cistos epidérmicos, pseudoacantose nigricante, queratoses seborreicas, nevos melanocíticos e manchas lentiginosas.

  15. 关于肢端肥大症治愈标准的共识%A consensus on criteria for cure of acromegaly

    赵红燕

    2010-01-01

    @@ 2009年肢端肥大症共识工作组(Acromegaly Consensus Group)制定了肢端肥大症的最新诊疗指南.同年4月,该工作组对2000年以来发表的治疗方案及治愈标准进行修改,并制定了新的肢端肥大症治愈标准.

  16. Medical Treatment for Acromegaly does not Increase the Risk of Central Adrenal Insufficiency: A Long-Term Follow-Up Study.

    Ceccato, F; Lizzul, L; Zilio, M; Barbot, M; Denaro, L; Emanuelli, E; Alessio, L; Rolma, G; Manara, R; Saller, A; Boscaro, M; Scaroni, C

    2016-08-01

    Central adrenal insufficiency (CAI) in acromegaly may be related to pituitary adenoma or induced by various medical treatments, transsphenoidal neurosurgery (TNS) or radiotherapy (RT), alone or combined. We assessed the role of all available treatments for acromegaly in inducing CAI. We retrospectively studied 97 patients. CAI was diagnosed if morning serum cortisol was <138 nmol/l, or if its response was inadequate in the low-dose short synacthen test. Seventy-four subjects underwent TNS (and 17 of whom also underwent RT), and 23 were on primary medical therapy: overall we diagnosed 21 cases of CAI. Duration of acromegaly, invasion of cavernous sinus, disease control, and type of medical treatment were much the same for patients with and without CAI, which was identified in 18% of patients (10/57) after one TNS, and in 53% (9/17) after RT (p=0.01); repeat surgery increased the risk of CAI (p=0.02). The risk of CAI onset during the follow-up was lower among patients treated with TNS or medical therapy than after RT (p=0.035). Medical treatment did not raise the risk of CAI, whereas a 5- and 4-fold higher risk of CAI was associated with repeat TNS and RT, respectively. Basal or stimulated cortisol levels were similar among acromegalic patients without CAI and matched controls with nonsecreting pituitary lesions. A significant proportion of patients with acromegaly developed CAI over time. While primary or secondary medical treatment did not contribute to the risk of CAI, repeat TNS and RT correlated with pituitary-adrenal axis impairment.

  17. 垂体生长激素瘤合并甲状腺高功能腺瘤一例报告并文献复习%Association of toxic thyroid adenoma with acromegaly: one case report and literature review

    谷伟军; 窦京涛; 杨国庆; 吕朝晖; 巴建明; 母义明; 陆菊明; 潘长玉

    2012-01-01

    Hyperthyroidism and acromegaly formed an unusual association.An acromegaly patient with a toxic thyroid adenoma was reported here,including clinical features,treatment,and final outcomes.The association of thyroid disease with acromegaly was reviewed.%生长激素瘤合并甲状腺高功能腺瘤临床较为罕见,本文报道1例垂体生长激素瘤合并甲状腺高功能腺瘤患者的临床特点、治疗、转归,并对两者关系的近期国内外研究进行复习.

  18. Concentrations of the acute phase reactants high-sensitive C-reactive protein and YKL-40 and of interleukin-6 before and after treatment in patients with acromegaly and growth hormone deficiency

    Andreassen, Mikkel; Vestergaard, Henrik; Kristensen, Lars Østergaard

    2007-01-01

    Acromegaly is accompanied by increased cardiovascular mortality and a cluster of proatherogenic risk factors. In the general population, ischaemic heart disease (IHD) is associated with elevated levels of inflammatory markers. The acute phase reactant (APR) C-reactive protein (CRP) has been...... reported to be reduced in acromegaly and increase after treatment, suggesting that excess of GH/IGF-I could have anti-inflammatory effects. This is in accordance with results obtained in patients with growth hormone deficiency (GHD), where increased levels of CRP have been reported....

  19. Acromegaly With Cardiomyopathy, Cardiac Thrombus and Hemorrhagic Cerebral Infarct: A Case Report of Therapeutic Dilemma With Review of Literature

    Mendoza, Erick; Malong, Chandy Lou; Tanchee-Ngo, Mary Jane; Mercado-Asis, Leilani

    2015-01-01

    Introduction: Cardiomyopathy with congestive heart failure (CHF) is a rare complication of growth hormone (GH)-secreting pituitary adenoma occurring in 3% of cases. We report a case of acromegaly complicated not only by CHF but also by the presence of intracardiac thrombus and cardioembolic stroke with hemorrhagic formation. Case Presentation: A 46-year-old Filipino female presented with amenorrhea, progressive coarsening of facial features, deepening of voice and enlargement of digits. She experienced easy fatigability, orthopnea and bipedal edema. The cardiac apex beat was sustained and displaced. Growth hormone was nonsuppressible. Cranial magnetic resonance imaging showed pituitary macroadenoma with hemorrhage. Incidentally, there was a left frontal lobe cortical infarct with hemorrhagic component. The echocardiogram demonstrated cardiomyopathic changes with a left ventricular thrombus. Conclusions: The primary treatment for GH-producing adenoma is surgery; however, this patient has high surgical risk from her severe cardiomyopathy. Radiotherapy poses a greater risk because of increased cerebrovascular mortality. Somatostatin receptor ligands are significantly associated with improvement of cardiovascular and hemodynamic parameters. Dopamine agonists must be considered regardless of prolactin level and immunostaining. The risks and benefits of any treatment must be emphasized in the presence of conflicting clinical features such as in the case reported. PMID:25926851

  20. Altered circadian blood pressure profile in patients with active acromegaly. Relationship with left ventricular mass and hormonal values.

    Pietrobelli, D J; Akopian, M; Olivieri, A O; Renauld, A; Garrido, D; Artese, R; Feldstein, C A

    2001-09-01

    To determine the relationships between the circadian blood pressure profile and left ventricular mass, hormonal pattern and insulin sensitivity indices in patients with active acromegaly, ambulatory 24-h blood pressure monitoring (ABPM) was recorded in 25 subjects (47.0 +/- 15.1 years, range 23-72). Serum growth hormone (GH) and insulin-like growth factor-1, fasting and mean plasma glucose and insulin during oral glucose tolerance test (OGTT), insulinogenic index, the sum of the plasma insulin levels and the homeostasis model insulin resistance index (Homa's index) were determined. Left ventricular mass index (LVMI) was calculated from two-dimensional guided M-mode echocardiogram. The prevalence of hypertension was 56% (n = 14) and 40% (n = 10) according to sphygmomanometric measurements and ABPM, respectively. Non-dipping profile was observed in six of 10 hypertensives and in six of 15 normotensives. Serum growth hormone, fasting glucose, the area under the serum insulin curve and LVMI were higher for acromegalics with non-dipping profile than for dippers (all of them, P regulation in essential hypertension deserves further studies.

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

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

    2014-01-01

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

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

    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.

  3. Pituitary apoplexy causing spontaneous remission of acromegaly following long-acting octreotide therapy: a rare drug side effect or just a coincidence

    Kumar, Sunil; Sharma, Shruti

    2016-01-01

    Pituitary apoplexy is characterized by abrupt onset of haemorrhage or non-haemorrhagic infarction of a pituitary adenoma. The clinical features include acute onset severe headache, visual field defects, meningeal irritation, ophthalmoplegia and hypopituitarism. The pituitary apoplexy may be clinically silent in ∼25% of patients. We report a case of acromegaly due to pituitary macroadenoma. The patient was started on long-acting octreotide therapy. On 3-month follow-up, the patient showed clinical and biochemical remission and the magnetic resonance imaging (MRI) of the brain showed subclinical haemorrhage and resolution of tumour. The octreotide therapy was stopped. On 6-month follow-up, the patient was still in remission and the MRI of brain revealed non-enhancing mixed intensities haemorrhagic and cystic areas of the pituitary gland. In our patient, whether spontaneous remission of acromegaly due to subclinical pituitary haemorrhage was coincidental or due to long-acting octreotide therapy is still a dilemma. We report this case because of rarity and clinical importance of this unusual occurrence. PMID:27123308

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

    Connock Martin J

    2009-10-01

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

  5. Profile of pegvisomant in the management of acromegaly: an evidence based review of its place in therapy

    Bernabeu I

    2015-02-01

    . Involved mechanism is currently unknown. New modalities of treatments by the combined use of PEG with somatostatin analog or cabergoline have been developed with promising results. Recently, two clinical guidelines written to optimize the use of these treatment modalities and to monitor possible adverse events have been published. Keywords: acromegaly, pegvisomant, pituitary tumor, somatostatin analogs, cabergoline, IGF-I

  6. Effects of nomifensine, an inhibitor of endogenous catecholamine re-uptake, in acromegaly, in hyperprolactinaemia, and against stimulated prolactin release in man.

    Scanlon, M F; Gomez-Pan, A; Mora, B; Cook, D B; Dewar, J H; Hildyard, A; Weightman, D R; Evered, D C; Hall, R

    1977-01-01

    1. Nomifensine, an inhibitor of endogenous catecholamine re-uptake, did not affect the growth hormone (GH) or prolactin levels in patients with acromegaly or hyperprolactinaemia. It does not, therefore, have any therapeutic role in these conditions at the dosage used in this study. 2. It had no effect on thyrotrophin-releasing hormone (TRH)-induced thyrotrophin (TSH) or prolactin release in males, yet caused marked suppression of monoiodotyrosine (MIT)-induced prolactin release in males but not in females. 3. The significant suppression of MIT-induced prolactin release in males is likely to reflect the dopamine (DA) agonist activity of the drug and its lack of effect in the other situations tested could be dose related. 4. It is proposed that the difference in male and female patterns of prolactin response to MIT after nomifensine, could be due to a "damping" effect of oestrogen on the hypothalamic dopaminergic system.

  7. Dynamic evaluation of growth hormone (GH) and prolactin (hPRL) secretion in active acromegaly with high and low GH output.

    Tolis, G; Kovacs, L; Friesen, H; Martin, J B

    1975-02-01

    Ten patients with active acromegaly were studied. In 9 plasma GH levels failed to suppress after glucose (OGTT), in 8 an increase in serum GH occurred after thyrotropin releasing hormone (TRH). After L-Dopa, 4 patients showed no change in serum GH, 3 exhibited a decrease and in 3 an increase in serum hGH occurred. With a combined insulin (ITT) and arginine (ATT) test, 2 patients exhibited an increase in hGH, and in 6 no change occurred. Fasting serum GH concentration was less than 11 ng/ml in 5 patients. Basal prolactin (hPRL) levels were normal in all patients including two with galactorrhea. L-Dopa suppressed and TRH stimulated hPRL secretion in all, but the responses which were seen were subnormal. Hydrocortisone infusion in two acromegalics did not affect the prolactin induced increase after TRH but blunted the GH increase after TRH.

  8. Myocardial involvement in acromegaly.

    van den Heuvel, P A; Elbers, H R; Plokker, H W; Bruschke, A V

    1984-10-01

    We recently encountered a case of an acromegalic patient with congestive heart failure and secondary cardiomyopathy. Cardiac biopsies were available in addition to autopsy material. This enabled us to perform electron microscopical studies which have hitherto not been reported. This may help to clarify the relationship between these two conditions and reveal specific characteristics.

  9. [Effective treatment with constant subcutaneous infusion of octreotide in a patient with acromegaly associated with diabetic pre-coma and diabetes insipidus].

    Abe, T; Ohguni, S; Tanigawa, K; Kato, Y

    1994-11-20

    A 58-year-old woman was admitted to our hospital for impaired consciousness, hyperglycemia and bitemporal hemianopsia. She was diagnosed as having NIDDM one year ago and was treated with diet and glibenclamide (1.25 mg/day) for 6 months. However, she stopped her medical treatment one month ago and then polydipsia and general fatigue were manifested. She was admitted to a hospital five days ago at which time hyperglycemia (405 mg/dl) and anemia (Hb8.0g/dl) were detected. She was transferred to our hospital for control of blood glucose and further examination of bitemporal hemianopsia. She showed typical acromegalic features including enlargement of the nose, lips and tongue, increased heel pad and acral growth. Conscious disturbance was cured by the infusion of saline and the administration of insulin. Endoscopy revealed an active gastric ulcer (A1). Endocrine data disclosed increased GH levels in plasma and urine, whereas plasma IGF-1 levels were low. Plasma GH paradoxically increased following the administration of TRH. A water deprivation test showed an impaired increase in urinary osmolarity, indicating partial central diabetes insipidus (DI). MRI with Gd-contrast revealed a macroadenoma which progressed toward suprasella. She was diagnosed as having acromegaly, partial DI and probable hyperosmolar hyperglycemic nonketotic diabetic pre-coma. Polyuria (5-101/day) due to partial DI was controlled by the administration of DDAVP (10 micrograms/day). The constant subcutaneous administration of octreotide (240 micrograms/day) resulted in normal plasma GH levels and a marked shrinkage of the pituitary tumor. The pituitary tumor was finally removed by the transsphenoidal approach following treatment with octreotide for 4 months. HE staining of the pituitary tumor showed atrophic and acidophilic cells surrounded by hyaloid connective tissue. After the surgery, plasma GH levels were normalized and complications were cured. In conclusion, this is a very rare case of

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

    Rochira Vincenzo

    2012-01-01

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

  11. Improvement of left ventricular hypertrophy and arrhythmias after lanreotide-induced GH and IGF-I decrease in acromegaly. A prospective multi-center study.

    Lombardi, G; Colao, A; Marzullo, P; Biondi, B; Palmieri, E; Fazio, S

    2002-12-01

    We report the results of a prospective Italian multi-center study of the effects of lanreotide, a slow-release somatostatin analog, on left ventricular morphology and function and on the prevalence of ventricular arrhythmic events in 19 patients with active, newly diagnosed, uncomplicated acromegaly. Cardiac features were evaluated with Doppler-echocardiography and 24-h Holter ECG monitoring at baseline and after 6 months of lanreotide therapy. Fifteen patients (78.9%) had left ventricular hypertrophy. Lanreotide treatment significantly decreased the left ventricular mass (127.8+/-6.9 vs 140.7+/-7.1 g/m2, paffect systolic function, whereas it increased the Doppler-derived early-to-late mitral flow velocity, (E/A) ratio, of early-to-late trans-mitral flow velocity (1.34+/-0.1 vs 1.09+/-0.06, p=0.001). Stroke volume was slightly but not significantly increased after treatment, whereas systolic BP was significantly higher (134+/-14 vs 129+/-13 mmHg, p50/24 h) occurred in 16.6% of patients and were unaffected by treatment. Differently, ventricular premature beats (>50/24 h) occurred in 33.3% of patients before treatment vs 16.5%, after treatment. In conclusion, lanreotide reduced the left ventricular mass, and improved ventricular filling and ventricular arrhythmic profile.

  12. A probable case of gigantism/acromegaly in skeletal remains from the Jewish necropolis of "Ronda Sur" (Lucena, Córdoba, Spain; VIII-XII centuries CE).

    Viciano, Joan; De Luca, Stefano; López-Lázaro, Sandra; Botella, Daniel; Diéguez-Ramírez, Juan Pablo

    2015-01-01

    Pituitary gigantism is a rare endocrine disorder caused by hypersecretion of growth hormone during growing period. Individuals with this disorder have an enormous growth in height and associated degenerative changes. The continued hypersecretion of growth hormone during adulthood leads to acromegaly, a condition related to the disproportionate bone growth of the skull, hands and feet. The skeletal remains studied belong to a young adult male from the Jewish necropolis of "Ronda Sur" in Lucena (Córdoba, Spain, VIII-XII centuries CE). The individual shows a very large and thick neurocranium, pronounced supraorbital ridges, an extremely prominent occipital protuberance, and an extremely large and massive mandible. Additional pathologies include enlargement of the vertebral bodies with degenerative changes, thickened ribs, and a slight increased length of the diaphysis with an increased cortical bone thickness of lower limbs. Comparative metric analysis of the mandible with other individuals from the same population and a contemporary Mediterranean population shows a trend toward acromegalic morphology. This case is an important contribution in paleopathological literature because it is a rare condition that has not been widely documented in ancient skeletal remains.

  13. 肢端肥大症术前使用生长抑素类似物的经济学评价研究%Pharmacoeconomic Evaluation on the Preoperative Treatment of Acromegaly with Somatostatin Analogues in Shanghai

    何江江; 李益明; 庄妍; 吴蔚; 刘晓霞; 金佳; 郑红淑; 朱国兰; 胡善联

    2014-01-01

    通过回顾性临床研究,开展生长抑素类似物(兰瑞肽和奥曲肽)在上海地区用于肢端肥大症术前治疗的药物经济学评价。收集到上海某三甲医院2009年1月1日-2013年6月30日的89例肢端肥大症患者的病史档案资料。研究结果显示,兰瑞肽和奥曲肽用于中国人群的临床疗效(IGF-I水平)控制达标情况相当,且与纯手术组不存在统计学差异;药品干预组的治疗费用明显高于纯手术组,但在干预组中,兰瑞肽组相对于奥曲肽组具有一定的成本优势。%This study evaluated the pharmacoeconomic effect of preoperative treatment of acromegaly in Shanghai by using Octreotide and Lanreotide through retrospective clinical study. The medical records of 89 acromegaly patients in a sampling hospital were collected during January 1, 2009 to June 30, 2013. Both Lanreotide and Octreotide were proven to be effective and fairly equivalence in lowering the level of IGF-I in the preoperative treatment of Chinese patients with acromegaly, and there was no signiifcant difference from the sole surgery group. The costs of treatment per patient of the sole surgery group was obviously lower than those of the drug intervention groups, however, the Lanreotide group had a cost-effective advantage over the Octreotide group.

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

    Classen Carl

    2012-01-01

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

  15. Acromegalia: aspectos diagnósticos e terapêuticos: análise de 18 casos Acromegaly: diagnostic and therapeutic aspects. analysis of 18 cases

    Flávio Freinkel Rodrigues

    1997-01-01

    Full Text Available Os autores fizeram estudo retrospectivo de 18 pacientes com acromegalia, que foram diagnosticados, tratados e acompanhados pelos Serviços de Endocrinologia e Neurocirurgia do Hospital Universitário Clementino Fraga Filho da Universidade Federal do Rio de Janeiro, no período de 1979 a 1985. A média de idade dos pacientes foi 43,2 anos (variação entre 15 e 63, as queixas clínicas iniciais se deveram principalmente às alterações somáticas (83,33%, em 50% dos casos tivemos manifestações secundárias à compressão tumoral e em 53,33% havia alterações neuro-oftalmológicas. A comprovação de hipersecreção de hormônio de crescimento foi feita por dosagens hormonais basais e por testes dinâmicos. A avaliação neuro-radiológica evidenciou expansão suprasselar em 61,11% dos casos e em 100% dos casos o acesso cirúrgico foi por via transesfenoidal. O objetivo principal deste estudo foi o de estabelecer critérios para o diagnóstico, discutir a conduta terapêutica e avaliar os resultados obtidos, comparando-os com outras séries da literatura.The authors present a retrospective study of a series of eighteen patients with acromegaly diagnosed, treated and followed by the Endocrinology and Neurosurgery Services of the Hospital Universitário Clementino Fraga Filho of the Federal University of Rio de Janeiro. The average age of the patients was 43.2% years (varying between 15 and 63. Initial complaints were mainly due to somatic alterations in 83.33%; half the cases had manifestations secondary to tumor compression and 53.33% had neuro-ophtalmological alterations. Hypersecretion of growth hormone was demonstrated by basal hormone determinations and dynamic tests. Neuroradiological assessment showed supraselar expansion in 61.11% of cases. Surgical approach was transsphenoidal in all cases. The main objetive of this study was to establish diagnostic criteria, discuss the therapeutic conduct and evaluate the results obtained, comparing

  16. The sensitivity of growth hormone and prolactin secretion to the somatostatin analogue SMS 201-995 in patients with prolactinomas and acromegaly.

    Lamberts, S W; Zweens, M; Klijn, J G; van Vroonhoven, C C; Stefanko, S Z; Del Pozo, E

    1986-08-01

    The somatostatin analogue SMS 201-995 has recently been shown to be effective in suppressing GH secretion in most acromegalic patients. In the present study it was investigated whether PRL release in prolactinoma and acromegalic patients might also be sensitive to SMS 201-995 and whether co-secretion of PRL in acromegaly plays a role in determining the sensitivity of GH secretion to SMS 201-995. The s.c. administration of 50 micrograms SMS 201-995 did not affect high plasma PRL levels in four microprolactinoma patients. Therapy of one of these patients for 3 d with 50 micrograms three times a day also did not affect PRL levels. The single administration of 50 micrograms SMS 201-995 in 22 acromegalic patients lowered plasma GH levels for 2-6 h to less than 5 micrograms/l in 14 patients and to less than 50% of control values in 16 patients. In 18 of these 22 patients the immunohistochemical picture of the pituitary tumour was known. Eleven patients had pure GH-containing tumours and in seven patients there were mixed GH/PRL-containing tumours. In two of these latter patients there was evidence for GH and PRL being secreted by the same tumour cells. The sensitivity of GH secretion to SMS 201-995 did not differ between the patients with pure GH or mixed GH/PRL-containing adenomas. Plasma PRL levels were not affected by SMS 201-995 in the patients with pure GH-secreting tumours, but were significantly suppressed in four of the seven patients with mixed GH/PRL containing tumours. Chronic treatment for 16 weeks of one patient with a mixed GH/PRL-containing tumour with SMS 201-995 (100 micrograms three times a day) resulted in normalization of both the increased GH and PRL levels. It is concluded that SMS 201-995 does not affect tumorous PRL secretion in patients with pure prolactinomas. In acromegalic patients with mixed GH/PRL-containing tumours PRL secretion in some patients is sensitive to SMS 201-995, making these patients good candidates for chronic treatment with

  17. Clinical features and natural course of acromegaly in patients with discordance in the nadir GH level on the oral glucose test and the IGF-1 value at 3 months after adenomectomy.

    Kinoshita, Yasuyuki; Tominaga, Atsushi; Usui, Satoshi; Arita, Kazunori; Sakoguchi, Tetsuhiko; Sugiyama, Kazuhiko; Kurisu, Kaoru

    2016-04-01

    Discordant GH and IGF-1 levels after adenomectomy are well recognized in acromegalics. The aim of this study was to evaluate the clinical features and natural course of postoperative acromegaly associated with discordant GH and IGF-1 levels over a postoperative period. A total of 69 acromegalics underwent surgery with at least 1 year of follow-up and received 75-g oral glucose tolerance tests (OGTTs) at 3 months postoperatively. The patients were categorized into four groups according to the postoperative nadir GH levels and IGF-1 levels: controlled group (normal GH and normal IGF-1), high-IGF-1 group (normal GH and high IGF-1), high-GH group (high GH and normal IGF-1), and uncontrolled group (high GH and high IGF-1). The incidence of discordant GH and IGF-1 levels was 27.5%: high-IGF-1 group = 10.1% (n = 7) and high-GH group = 17.4% (n = 12). All patients in the high-IGF-1 group exhibited a decline in the IGF-1 level after surgery, with normalization observed in 71.4% of the patients without additional treatment (median 23 months). These subjects had preoperatively high IGF-1 levels despite not demonstrating higher GH levels than the patients in the controlled group. On the other hand, four patients in the high-GH group exhibited an elevated nadir GH level higher than 1.0 μg/L on repeated OGTTs after 3 months, and one patient experienced a recurrence of acromegaly. Patients in the high-IGF-1 group require no additional treatments, and their IGF-1 levels are likely to normalize within a few years. However, patients in the high-GH group should be carefully followed due to the possibility of recurrence.

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

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

    2015-01-10

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

  19. Interpretation of medical guidelines for clinical practice for the diagnosis and treatment of acromegaly - 2011 update%2011年美国内分泌医师学会肢端肥大症诊疗临床实践指南解读

    田丹; 岑晶; 顾锋

    2012-01-01

    Acromegaly is a rarely encountered endocrine disease featured by growth hormone hypersecretion arid multisystem involvement. Subjects often had to resort to multiple departments and may even be misdiagnosed as a result of insidious onset and non-specific clinical manifestations. Pituitary macroadenoma has not been uncommon in patients with newly diagnosed acromegaly. The cardiovascular and respiratory complications may lead to markedly reduced quality of life, rendering it difficult to cure and an increasing mortality rate. That most studies have addressed biochemical diagnostic criteria and individualized treatment protocol of acromegaly has resulted in an optimal amelioration in terms of biochemical parameters via the treatment using medications,particularly the combination with surgery. More attention should be attached to acromegaly leading to improved capacity of the diagnosis and treatment.%肢端肥大症是一种较为少见的内分泌疾病,以生长激素分泌过多伴多系统受累为特征.因其隐匿且非特异的临床表现,患者常常往返于多个科室而不被临床医师重视甚至误诊.多数患者在明确诊断时常表现为垂体大腺瘤,合并的心血管系统、呼吸系统等相关并发症严重影响患者的生活质量,使治疗难度增加同时病死率升高.目前许多研究关注于肢端肥大症的生化诊断和缓解标准以及个体化的治疗方案,而药物治疗尤其是药物联合手术治疗使部分难治性肢端肥大症患者获得了最大程度的生化缓解.临床上需要对该病引起重视,提高诊断与治疗水平.

  20. Primary medical therapy for acromegaly: an open, prospective, multicenter study of the effects of subcutaneous and intramuscular slow-release octreotide on growth hormone, insulin-like growth factor-I, and tumor size.

    Bevan, J S; Atkin, S L; Atkinson, A B; Bouloux, P-M; Hanna, F; Harris, P E; James, R A; McConnell, M; Roberts, G A; Scanlon, M F; Stewart, P M; Teasdale, E; Turner, H E; Wass, J A H; Wardlaw, J M

    2002-10-01

    Conventional surgery and radiotherapy for acromegaly have limitations. There are few data on the use of the somatostatin analog octreotide (Oct) as primary medical therapy. An open prospective study of 27 patients with newly diagnosed acromegaly was conducted in nine endocrine centers in the United Kingdom. Twenty patients had macroadenomas, and 7 had microadenomas. For the first 24 wk (phase 1), patients received sc Oct in an initial dose of 100 microg, 3 times daily, increased to 200 micro g three times daily after 4 wk in the 13 patients whose mean serum GH remained greater than 5 mU/liter (2 microg/liter). Five-point GH profiles were performed at 0, 4, 12, and 24 wk, and high resolution pituitary imaging using a standard protocol was performed at 0, 12, and 24 wk (magnetic resonance imaging in 25 patients and computed tomography in 2). Tumor dimensions and volumes were calculated by a central, reporting neuroradiologist, and the results were audited by a second, independent neuroradiologist. After 24 wk, 15 patients proceeded to phase 2 of the study with a direct switch to monthly injections of the depot formulation of Oct, Sandostatin long-acting release (Oct-LAR). Further GH profiles were performed at 36 and 48 wk, and pituitary imaging was performed at 48 wk. The median pretreatment serum GH concentration was 30.7 mU/liter (range, 6.7-141.4). During sc Oct, serum GH fell to less than 5 mU/liter in 9 patients (38%), and IGF-I fell to normal in 8 patients (33%). All 27 tumors shrank during sc Oct; for microadenomas the median tumor volume reduction was 49% (range, 12-73), and for macroadenomas it was 43% (range, 6-92). After 24 wk of Oct-LAR (end of phase 2), the GH level was less than 5 mU/liter in 11 of 14 patients (79%), and IGF-I was normal in 8 of 15 patients (53%). In the 15 patients given Oct-LAR (10 macroadenomas), wk 48 scans showed a further overall median tumor volume reduction of 24%. At the end of the study 79% of patients had mean serum GH levels

  1. Transsphenoidal endoscopic surgery for acromegaly: a clinical analysis of 210 cases%经鼻蝶窦内镜手术治疗肢端肥大症210例临床分析

    张艳阳; 周涛; 孟祥辉; 许百男

    2015-01-01

    目的 探讨经鼻蝶窦内镜手术治疗肢端肥大症的疗效.方法 回顾性分析2007年1月至2014年1月经鼻蝶窦内镜手术的210例肢端肥大症患者的临床资料.术前手术风险较高的患者使用长效生长抑素类似物奥曲肽,术中结合应用高场强术中核磁共振和神经导航,术后随访头颅MRI及内分泌包括生长激素和类胰岛素样生长因子-1等生化指标,肿瘤残留及内分泌未缓解病例根据情况选择奥曲肽或放射外科治疗.结果 男129例,女81例;年龄21 ~ 67岁,平均40.7岁.术前MRI显示肿瘤最大径>1 em 183例(87.1%).4例术前应用奥曲肽.肿瘤全切171例(81.4%)例,次全切除22例(10.5%),部分切除17例(8.1%).本组无手术相关死亡病例.术后内分泌缓解148例(70.5%),术前平均生长激素为27 ng/ml(4~150 ng/ml)降至术后5 ng/ml(0~ 32 ng/ml).184例患者获得随访,随访平均27.5个月(3~ 84个月).随访中,161例(87.5%)患者自觉症状改善并参加日常工作和学习.在术后肿瘤残留及内分泌未缓解的病例中,34例术后应用奥曲肽,29例接受放射外科治疗.结论 经鼻蝶窦内镜手术是肢端肥大症的首选治疗方案.术后要定期复查头颅MRI及内分泌.奥曲肽及放射外科治疗可以控制残留肿瘤并改善内分泌.%Objective To investigate the efficacy of transsphenoidal endoscopic surgery for acromegaly.Methods The clinical data of 210 consecutive patients with acromegaly operated with transsphenoidal endoscopic surgery from January 2007 to January 2014 were analyzed retrospectively.The patients with preoperative higher surgical risk used long-acting somatostatin analogue octreotide.Surgery was conducted in combination with intraoperative high field magnetic vesonance imaging and neuronavigation,postoperative head MRI and endocrine were followed up,including biochemical indicators,such as growth hormone and insulin-like growth factor-1.The patients with residual tumor

  2. Efficacy of somatostatin analogues in combination with surgery in patients with acromegaly:a review of 23 cases%长效生长抑素类似物联合手术治疗肢端肥大症疗效分析

    岑晶; 顾锋; 金自孟; 伍学焱; 朱惠娟; 李乃适; 茅江峰; 段炼; 岳欣

    2012-01-01

    Objective To study the efficacy of somatostatin analogues (SSA) with surgery combined in patients with acromegaly. Methods We performed a retrospective study on the clinical profiles in 23 patients with pathologic ally-confirmed acromegaly who were treated with presurgical SSA in combination with transsphenoidal tumor resection and underwent long-term followed-ups in the department of endocrinology, Peking Union Hospital between January 1st,2005 and December 31 st,2008. Results Treatment with SSA in patients with acromegaly for >3 months led to marked reduction in the nadir of growth hormone (GH) (P < 0. 01) and shrinking of tumor volume ( P = 0. 012). Biochemically controlled group ( GH < 0. 047 nmol/L) yielded considerbly smaller tumor size than uncontrolled group (GH ≥0. 047 nmol/L) (P = 0. 015) prior to the surgery. In addition,both initial and final GH nadirs were reduced postoperatively (P=0. 002 and 0. 018.respectively) . Conclusion SSAs in patients with acromegaly may be associated with improved efficacy of surgery as a result of reduced GH level and shrinked tumor volume prior to surgery. Preoperative detection of GH nadir via OGTT may be applied to assess the prognosis of patients with acromegaly.%目的 评价初始长效生长抑素类似物联合手术治疗肢端肥大症的临床疗效.方法 回顾性分析2005-2008年在北京协和医院内分泌科收治并定期随访的初始长效生长抑素类似物(SSA)治疗联合经蝶手术切除垂体腺瘤并经病理证实的23例肢端肥大症患者病历资料.结果 肢端肥大症患者长效SSA治疗(≥3个月)后生长激素(GH)谷值明显降低(P<0.01),肿瘤体积缩小(P=0.012).术前生化控制组(GH<0.047 nmol/L)较未控制组(GH≥0.047 nmol/L)肿瘤体积小(P=0.015),术后初次(P=0.002)及末次随访(P=0.018)GH谷值低.结论 肢端肥大症患者术前应用长效SSA治疗可降低术前GH水平、缩小肿瘤体积,是提高手术疗效的原因之一;术前口服葡

  3. Effects of antagonists of growth hormone-releasing hormone (GHRH) on GH and insulin-like growth factor I levels in transgenic mice overexpressing the human GHRH gene, an animal model of acromegaly.

    Kovacs, M; Kineman, R D; Schally, A V; Zarandi, M; Groot, K; Frohman, L A

    1997-11-01

    Transgenic mice overexpressing the human GH-releasing hormone (hGHRH) gene, an animal model of acromegaly, were used to investigate the effects of potent GHRH antagonists MZ-4-71 and MZ-5-156 on the excessive GH and insulin-like growth factor I (IGF-I) secretion caused by overproduction of hGHRH. Because metallothionein (MT)-GHRH mice express the hGHRH transgene in various tissues, including the pituitary and hypothalamus, initial experiments focused on the effectiveness of the GHRH antagonists in blocking basal and stimulated GH secretion from pituitary cells in vitro. Both MZ-4-71 and MZ-5-156 suppressed basal release of GH from superfused MT-GHRH pituitary cells, apparently by blocking the action of endogenously produced hGHRH. In addition, these antagonists effectively eliminated the response to stimulatory action of exogenous hGHRH(1-29)NH2 (30 and 100 nM). To ascertain whether MZ-4-71 and MZ-5-156 could antagonize the effect of hGHRH hyperstimulation in vivo, each antagonist was administered to MT-GHRH transgenic mice in a single iv dose of 10-200 microg. Both compounds decreased serum GH levels in transgenic mice by 39-72% at 1 h after injection. The inhibitory effect of 50 microg MZ-5-156 was maintained for 5 h. Twice daily ip administration of 100 microg MZ-5-156 for 3 days suppressed the highly elevated serum GH and IGF-I concentrations in transgenic mice by 56.8% and 39.0%, respectively. This treatment also reduced IGF-I messenger RNA levels in the liver by 21.8% but did not affect the level of GH messenger RNA in the pituitary. Our results demonstrate that GHRH antagonists MZ-4-71 and MZ-5-156 can inhibit elevated GH levels caused by overproduction of hGHRH. The suppression of circulating GH concentrations induced by the antagonists seems to be physiologically relevant, because both IGF-I secretion and synthesis also were reduced. Our findings, showing the suppression of GH and IGF-I secretion with GHRH antagonists, suggest that this class of analogs

  4. Case-control study of expansion of heart cavity and great vessel diameters in acromegaly%肢端肥大症患者心腔及大血管内径变化的病例对照研究

    郭晓鹏; 高路; 张硕; 李一琳; 方理刚; 幸兵

    2015-01-01

    目的 探讨肢端肥大症患者的心房心室腔及大血管内径的变化情况及其相关危险因素.方法 选取2013年1至12月在北京协和医院神经外科确诊为肢端肥大症的92例患者(试验组),其中男37例,女55例,年龄19 ~67(41±13)岁,并根据试验组性别、年龄选取92例健康体检者作为对照组,采集两组患者相关病史、超声心动图及内分泌激素检查等信息进行对比分析,并采用二元logistic回归分析筛选与发生大血管内径扩大相关的危险因素,得出OR值及预测模型.结果 试验组患者的左室收缩末内径(LVESD)、左室舒张末内径(LVEDD)、左房前后径(LALD)、右室前后径(RVLD)、主动脉根部内径(AORD)、升主动脉内径(AA)及主肺动脉内径(MPA)均较对照组有明显扩大,P <0.05;其中试验组的大血管内径扩大者(ADE)与内径正常者的病程(月)对比差异有统计学意义(121 ±70比73±59,P<0.05,OR=1.011),建立回归方程为:logit (ADE)=-3.420 +0.011×病程.结论 肢端肥大症患者心腔及大血管内径较正常人有扩大,其中大血管内径扩大与病程长短有关,与GH、IGF-1高低无关,病程时间长是其危险因素.%Objective To investigate alternations of heart cavity and great vessel diameters and their risk factors in acromegalic patients.Methods Ninety-two cases,diagnosed as acromegaly,were selected as research subjects from January 2013 to December 2013 in Peking Union Medical College Hospital.There were 37 males and 55 females with an average age of 41.2 years (SD,12.5 years,range from 19 to 67 years old).Ninety-two sex and age matched healthy individuals were selected as controls.Disease history,echocardiography,endocrine hormone levels were collected to make contrastive analysis.We analyzed the related risk factors of great vessel diameters expansion with binary logistic regression analysis and finally obtained the OR (odd ratio) and predictive formula.Results Left ventricle

  5. Acromegaly and sleep apnea: cephalometric evaluations.

    Bruwier, A; Albert, A; Beckers, A; Limme, M; Poirrier, R

    2011-06-01

    Lateral teleradiography is a standard and quick examination. It has enabled us to define differences as regards to the craniofacial morphology between 20 acromegalic patients and 20 control subjects. The height of the mandibular ramus (from the posterior condyle point to the gonion point) increases significantly with the acromegalic patient and the cranial base angle (basion-superior tuberculum sellae-M point) is more extended. As acromegalic patients are more subject to sleep apnea (30% prevailing), the relationship between the amount of sleep apnea and hypopnea (AHI) in an acromegalic patient and his bone, tissue and hormone factors has been researched, in order to act on the causes of sleep apnea. It has emerged that confronting craniofacial bones and soft tissues factors enables a good prediction of the apnea and hypopnea index. Of course, we can find again the potential action of growth hormone (GH) but only in fifth place in importance order. The tongue, which increases in volume with the GH impact, is in a too short "lingual enclosure" (reduced length of the mandibular horizontal branch).

  6. 肢端肥大症%Acromegaly

    Belayet Hossain; William M Drake; 张化冰

    2006-01-01

    肢端肥大症是一种成年人中罕见、慢性、致残性疾病,其致残率和死亡率高。它几乎均由分泌生长激素(GH)的垂体腺瘤引起。在骨骺闭合前发病者可以引起垂体性巨人症。在未治疗的个体中生存年限大约减少10年。

  7. New Insights into Medical Treatment of Acromegaly

    S.J.C.M.M. Neggers (Bas)

    2011-01-01

    textabstractGiants and Acromegalics fascinated people, since ancient times. Historical artifacts, paintings, illustrations, photographs or articles have documented many. The earliest medical reports date back to 1516. In 1864 Verga was the first to describe an acromegalic in medical literature and c

  8. Metabolic aspects of acromegaly and its treatment.

    Quabbe, H J; Plöckinger, U

    1996-08-01

    Growth hormone (GH) affects virtually all facets of metabolism. This review concentrates on the effects of GH excess on carbohydrate, lipid, and bone metabolism, and on body composition. The effect of treatment with the somatostatin analog, octreotide, on the gastrointestinal-pancreatic axis is also discussed.

  9. Somatostatin analog treatment of acromegaly: new aspects.

    Lamberts, S W; del Pozo, E

    1988-01-01

    Ten acromegalics received daily doses of 200-300 micrograms of a long-acting somatostatin analog, SMS 201-995 (Sandostatin, SMS), for an average of 64 weeks. Basal mean GH values of 44 +/- (SE) 7.8 ng/ml had fallen into the normal range at the end of the observation period (mean 64 weeks). This effect was accompanied by a substantial drop in somatomedin-C values. Reduction of pituitary tumor size could be documented in 3 of 6 patients. Whereas SMS did not affect high plasma PRL in 4 microprolactinoma patients, lactotrophs turned sensitive to this agent in mixed GH/PRL tumors. In a comparative study between SMS and bromocriptine, the former normalized circulating GH in 10 of 17 acromegalics in an acute trial, whereas bromocriptine was effective in only 5. A combination of both substances was effective in 2 of 3 patients who were insensitive to single drug administration. Cultures of GH-secreting tumor cells showed a statistically significant hormone decrease in the medium when exposed to SMS. However, in some instances, a diminution of the GH contents of the tumor cells was also observed, presumably as the basis for intracellular breakdown and clinical tumor shrinkage.

  10. Hypopituitarism following pituitary irradiation for acromegaly

    Aloia, J.F.; Archambeau, J.O.

    1978-01-01

    Endocrine evaluation is reported in 8 acromegalic patients who received 5500 rad to the pituitary from a linear accelerator. There was a mean decrease in hGH levels of 72%. Plasma testosterone levels were low in 1 of the 6 male patients prior to pituitary irradiation and were below normal in all male patients on the final evaluation (3.1 +- 0.2 SD years postirradiation). Deficiency of TSH secretion developed in 2 patients following irradiation. This rather high incidence of postirradiation partial hypopituitarism was not anticipated and is thought to be related to radiation necrosis of the normal pituitary tissue which surrounds the adenoma.

  11. MRI characteristics of pituitary adenomas and relationships among the adenomas volume, serum GH levels and pathological immune responses in patients with acromegaly%肢端肥大症型垂体腺瘤MRI特点及其与血清GH水平、肿瘤GH表达水平的关系分析

    孔令胜; 姚维成; 栗世方; 贺昭忠; 成磊

    2014-01-01

    目的:探讨肢端肥大症型垂体腺瘤MRI特点及其与血清生长激素(GH)水平、肿瘤GH表达水平之间的关系。方法回顾性分析2008年1月~2013年6月采用经鼻蝶入路显微手术切除的84例肢端肥大症型垂体腺瘤患者的临床资料。结果MRI显示肿瘤可突破鞍膈向鞍上生长、突破鞍底向蝶窦内生长,鞍下侵犯指数[(2.35±0.69)cm]明显高于鞍上侵犯指数[(0.66±0.25) cm;P0.05)。结论肢端肥大症型垂体腺瘤有明显的向蝶窦内优先侵犯生长的趋势;其术前血清GH水平、肿瘤GH表达水平及瘤体大小之间无明显相关性。%Objective To explore the MRI characteristics of the pituitary adenomas and relationships among the adenomas volume, basic serum growth hormone (GH) level, pathological immune responses in the patients with acromegaly. Methods The clinical data of 84 acromegaly patients with pituitary adenomas, who underwent microsurgery via single-nostril transspheniodal approach from January, 2008 to June, 2013, were analyzed retrospectively. Results MRI showed that some tumors broke the sellar diaphragm into suprasellar regions or the sellar floor into the sphenoid sinuses. Infrasellar invasion index [(2.35±0.69) cm] was significantly higher than that [(0.66 ± 0.25) cm] of suprasellar invasion index (P0.05). Conclusions The pituitary adenomas volumes are insignificantly related to the preoperative basic serum GH level, and GH pathological immune response strength in the patients with cromegaly, in whom the pituitary adenomas trends to growth into the sphenoid sinuses.

  12. Effects of conventional high-voltage irradiation and proton irradiation as adjuncts to surgical measures in acromegaly with particular attention being given to the further course of illness with and without drug treatment. Effekt der konventionellen Hochvoltbestrahlung und der Protonenbestrahlung als Sekundaertherapie nach Operation bei Akromegalie unter Beruecksichtigung der medikamentoesen Nachbehandlung und der Spontanverlaeufe

    Lutz, B.S.

    1986-09-03

    Comparisons of effectiveness and side-effect levels were carried out between conventional high-voltage irradiation and proton irradiation being used as adjunctive treatment in 56 patients suffering from acromegaly, whose STH levels had failed to return to normal (STH > 4.5 ng/ml) after surgery. For the evaluation of the results it was also of importance, if the patients were subjected to drug treatment or allowed to recover spontaneously during follow-up. The two therapeutic strategies were found to be equally potent. In both patient groups, the decreases in STH achieved over 4.5 to 5 years averaged 80%. Proton irradiation was associated with a higher rate of injuries to the anterior lobe of the pituitary, while the damage caused by conventional irradiation mostly was of a more severe nature (like generalised attacks). On an average, the STH levels of patients under treatment with dopamine agonists were reduced by approx. 67% within three years. In the group of patients receiving no further treatment in spite of sustained STH elevations (spontaneous recoveries) after surgery, 76.5% were found to have normal STH basal values by that time. (ECB).

  13. Prediction of the pharmacological effect of octreotide in acromegaly by means of {sup 111}In-pentetreotide scintigraphy and calculation of a pituitary uptake index; Praediktion der pharmakologischen Wirkung von Octreotid bei Akromegalie mittels {sup 111}In-Pentetreotid-Szintigraphie und Berechnung eines hypophysaeren Uptake-Index

    Goerges, R. [Mainz Univ. (Germany). Klinik und Poliklinik fuer Nuklearmedizin; Cordes, U. [Arbeitsgemeinschaft Niedergelassener Endokrinologen, Mainz/Darmstadt (Germany); Engelbach, M. [Mainz Univ. (Germany). III. Medizinische Klinik und Poliklinik; Bartelt, K.M. [Arbeitsgemeinschaft Niedergelassener Endokrinologen, Mainz/Darmstadt (Germany); Haberern, G. [Mainz Univ. (Germany). Klinik und Poliklinik fuer Nuklearmedizin; Hey, O. [Mainz Univ. (Germany). Neurochirurgische Klinik und Poliklinik; Beyer, J. [Mainz Univ. (Germany). III. Medizinische Klinik und Poliklinik; Bockisch, A. [Mainz Univ. (Germany). Klinik und Poliklinik fuer Nuklearmedizin

    1997-06-01

    Aim: The aim of our prospective study was to optimize the determination of the pituitary somatostatin receptor status by means of 111-In-pentetreotide scintigraphy and to compare it intraindividually with the pharmacological effect of octreotide in active acromegaly. Methods: In n=22 patients with growth hormone (GH) secreting pituitary adenoma, 111-In-penetreotide scintigraphy was performed, and the specific radionuclide accumulation in the pituitary area (evaluation visually as well as semiquantitatively by means of ROI technique and calculation of various uptake indices) was correlated with the acute drop of GH after administration of 100 {mu}g octreotide s.c. (octreotide acute test). Results: The uptake index we propose (cts/pixel-ratio circular pituitary ROI: Irregular cerebrum ROI after background correction in the sagittal SPECT slice with maximum pituitary uptake 24 h p.i.) correlates best with the pharmacological effect (acute decrease of GH levels) of octreotide; its upper normal limit amounts of 3.5. Conclusion: As often the normal pituitary gland can be visualized scintigraphically, the purely visual differentiation between a normal and pathological receptor status sometimes is equivocal. A pituitary uptake index, calculated by means of a standardized ROI technique, facilitates this discrimination and so contributes to select possible responders for a treatment with octreotide. (orig.) [Deutsch] Ziel: Ziel unserer prospektiven Studie war die optimierte Bestimmung des hypophysaeren Somatostatin-Rezeptorstatus in der 111-In-Pentetreotid-Szintigraphie und der intraindividuelle Vergleich mit dem pharmakologischen Effekt von Octreotid bei florider Akromegalie. Methoden: Bei n=22 Patienten mit Wachstumshormon-(GH)-sezernierendem Hypophysenadenom wurde eine 111-In-Pentetreotid-Szintigraphie durchgefuehrt und die spezifische Nuklidakkumulation in der Hypophysenregion (Auswertung sowohl visuell als auch semiquantitativ mittels ROI-Technik und Berechnung

  14. Acromegalia: comparación de los niveles séricos de IGF-I por dos inmunoensayos y su correlación con la prueba de tolerancia oral a la glucosa Acromegaly: Comparison of serum IGF-1 levels measured by two immunoassays and their correlation with the Oral Glucose Tolerance Test

    L. Boero

    2010-12-01

    Full Text Available Introducción: La determinación de IGF-I en suero o plasma es una herramienta esencial en el diagnóstico y seguimiento de la acromegalia. Sin embargo, se deben tener presentes algunos inconvenientes en su medición por diferentes inmunoensayos. Objetivos: Evaluar dos inmunoensayos para la determinación de IGF-I y su correlación con el nadir de GH en el TTOG en pacientes acromegalicos. Materiales y métodos: Se analizaron 37 pacientes acromegálicos, 20 mujeres y 17 hombres. IGF-I fue determinada por Immulite 1000, (IMM y por IRMA (DSL. Se realizó el TTOG y se determinó glucosa y GH en todos los tiempos (basal, 30, 60, 90 y 120min. Se consideró respuesta normal un nadir de GH 1ng/ml se encontró en el 70%, con nadir de GH normal en el 13,5%. IGF-I normal con nadir >1ng/ml en el 2,7% y con nadir de GH normal en el 13,5%. El área bajo las curvas ROC no mostró diferencias significativas. Conclusiones: Los niveles de IGF-I determinados por IMM y DSL fueron significativamente diferentes mostrando un bias negativo para IMM. La mayoría de los valores del nadir de GH fueron consistentes con los niveles de IGF-I observándose una discrepancia en el 30% de los pacientes, estuvieran o no bajo tratamiento.Introduction: IGF-I determination in serum or plasma is an essential tool in the diagnosis and follow-up of acromegaly. Hepatic production of IGF-I is regulated by GH and circulates bound to several IGF-I binding proteins which extends its half life. IGF-I is not released in a pulsatile pattern and has no significant variability in 24 h. Objective: To evaluate two different methodologies in IGF-I levels determination and their correlation with GH nadir in OGTT in acromegalic patients. Material and methods: We analyzed 37 acromegalic patients, 20 women and 17 men, mean age was 45±12 years. IGF-I levels were assayed by Immulite 1000, DPC (IMM and DSL-5600 ACTIVE® IGF-I Coated-Tube IRMA (DSL and OGTTs (at baseline and at 30, 60, 90 and 120 minutes

  15. Skin morphological changes in growth hormone deficiency and acromegaly

    Lange, Merete Wolder; Thulesen, J; Feldt-Rasmussen, U

    2001-01-01

    To evaluate the histomorphology of skin and its appendages, especially eccrine sweat glands, in patients with GH disorders, because reduced sweating ability in patients with growth hormone deficiency (GHD) is associated with increased risk of hyperthermia under stressed conditions....

  16. Cardiovascular involvement in patients affected by acromegaly: an appraisal.

    Mosca, Susanna; Paolillo, Stefania; Colao, Annamaria; Bossone, Eduardo; Cittadini, Antonio; Iudice, Francesco Lo; Parente, Antonio; Conte, Sirio; Rengo, Giuseppe; Leosco, Dario; Trimarco, Bruno; Filardi, Pasquale Perrone

    2013-09-01

    Cardiovascular complications are frequent in acromegalic patients. Several studies reported increased prevalence of traditional cardiovascular risk factors and early development of endothelial dysfunction and of structural vascular alterations, with subsequent increased risk of coronary artery disease. Furthermore, chronic exposure to high levels of GH and IGF-I leads to the development of the so called "acromegalic cardiomyopathy", characterized by concentric biventricular hypertrophy, diastolic dysfunction and, additionally, by progressive impairment of systolic performance leading to overt heart failure. Cardiac valvulopathies and arrhythmias have also been documented and may concur to the deterioration of cardiac function. Together with strict control of cardiovascular risk factors, early control of GH and IGF-I excess, by surgical or pharmacological therapy, has been reported to ameliorate cardiac and metabolic abnormalities, leading to a significant reduction of left ventricular hypertrophy and to a consistent improvement of cardiac performance.

  17. The prevalence and characteristics of colorectal neoplasia in acromegaly.

    Renehan, A G; Bhaskar, P; Painter, J E; O'Dwyer, S T; Haboubi, N; Varma, J; Ball, S G; Shalet, S M

    2000-09-01

    An increased prevalence of colorectal neoplasia has been reported in acromegalic patients, and recommendations have been made for early colonoscopic screening and regular surveillance. This assumption, however, is frequently drawn from studies using selected control populations. To clarify colonoscopic management in these patients, we undertook a 2-center prospective screening colonoscopy study in 122 acromegalics (age range, 25-82 yr). In the absence of ideal age-matched controls, we calculated prevalence rates of occult adenocarcinomas and adenomas in the general population using cumulative data in the published literature from 8 autopsy studies (model 1, n = 3,559) and 4 screening colonoscopy studies (model 2, n= 810), applying linear regression models. Of the 115 patients with complete examinations, adenocarcinomas were discovered in 3 (2.6%), and at least 1 adenoma was found in 11, giving an overall prevalence of neoplasia of 12% (14 of 115). Prevalence rates for age bands 30-40, 40-49, 50-59, 60-69, and 70+ yr were 0%, 8%, 12%, 20%, and 21%, respectively. Compared with the 2 control models, the prevalence of occult colorectal cancer was not significantly increased (acromegalics vs. models 1 and 2, 2.6% vs. 2.3% and 0.9%), nor was there an increase in the prevalence of adenomas in any age band. Pathological characteristics showed some differences, in that adenomas in acromegalics tended to be right sided (68% vs. 57% and 56%), larger (for > or =10 mm, 27% vs. 13% and 9%), and of advanced histology (for tubulovillous, 27% vs. 4% and 22%). No associations were found between the presence of colonic neoplasia and the duration of disease, total GH exposure, cure status, and serum insulin-like growth factor I. This study has failed to demonstrate an increased prevalence of neoplasia in acromegalic patients compared with the expected prevalence in the general population and questions the need for an aggressive colonoscopic screening policy.

  18. Radial artery cannulation. Potential hazard in patients with acromegaly.

    Campkin, T V

    1980-10-01

    Using Allen's test, impaired ulnar artery circulation to one or both hands was detected pre-operatively in five out of ten acromegalic patients scheduled for transphenoidal hypophysectomy. Three of these patients also had symptoms of compression of the median nerve at the wrist (carpal tunnel syndrome). If ulnar flow is considered to be inadequate cannulation of a dorsalis pedis artery provides one possible alternative route for continuous measurement of the blood pressure during induced hypotension. In the three patients in this report in whom this vessel was cannulated no ischaemic complications in the foot were seen.

  19. Childhood acromegaly successfully treated with interstitial irradiation using Yttrium-90

    Rosenstock, J.; Doyle, F.H.; Mashiter, K.; Joplin, G.F. (Royal Postgraduate Medical School, Hammersmith Hospital, London (UK). Dept. of Medicine and Radiology); Hall, R. (Royal Victoria Infirmary, Newcastle upon Tyne (UK))

    1982-01-01

    A child with a growth hormone producing tumour presented at the age of 4 1/2 years. The onset of the disease was at 18 months of age. Treatment was given with three doses of interstitial irradiation using Yttrium-90 implants. There were no local complications from the procedures. Now, 11 years after diagnosis, she is asymptomatic, of normal appearance, and her height and the size of the pituitary fossa are normal. Growth hormone levels are almost normal, thyroid function is intact, and she is maintained on prednisone and sex hormones.

  20. Thyroid nodules in acromegaly: The role of elastography.

    Andrioli, M; Scacchi, M; Carzaniga, C; Vitale, G; Moro, M; Poggi, L; Fatti, L M; Cavagnini, F

    2010-09-01

    Sommario INTRODUZIONE: L’elastosonografia (US-E) è un utile strumento per la diagnosi del carcinoma tiroideo. Se nell’acromegalia lo struma multinodulare ha un’elevata prevalenza, ancora non vi è accordo sulla possibilità di un’aumentata prevalenza delle neoplasie maligne tiroidee. Obiettivi dello studio sono stati: a) valutare i noduli tiroidei nell’acromegalia con l’US-E; b) stabilire l’accuratezza di questa tecnica nel fornire informazioni sulla loro natura, usando l’esame citologico come parametro di riferimento. MATERIALI E METODI: L’US-E è stata eseguita in 25 pazienti acromegalici (7 guariti dalla chirurgia ipofisaria, 8 controllati dalla terapia medica, 10 con patologia attiva) che presentavano almeno un nodulo solido. Un totale di 90 noduli sono stati classificati secondo un punteggio elastosonografico (ES) in quattro classi di durezza: ES1 e ES2 per i noduli più elastici, ES3 e ES4 per quelli più duri. L’agoaspirato (FNAC) è stato effettuato nel 78.6% dei noduli ES 4 e nel 54,1% dei noduli ES 3. RISULTATI: Dei 90 noduli, 14 (15,5%) mostravano un ES 1, 25 (27,7%) un ES 2, 37 (41,3%) un ES 3 e 14 (15,5%) un ES 4. La prevalenza di noduli duri nei pazienti con acromegalia attiva (68,9%) era maggiore di quella osservata nei pazienti guariti (44,4%) e nei pazienti controllati (52,5%). Nell’intera popolazione di acromegalici la prevalenza di noduli duri (56,7%) era maggiore di quella riportata dalla letteratura nei pazienti non acromegalici con struma multinodulare (33,7%). Tutti i noduli sottoposti a FNAC sono risultati negativi per cellule tumorali maligne o per sospetta malignità. DISCUSSIONE: Questo studio ha dimostrato un’elevata prevalenza di noduli duri nell’acromegalia, soprattutto nei pazienti con patologia attiva. Tale prevalenza è maggiore di quella riportata in letteratura nei pazienti non acromegalici con patologia nodulare tiroidea. Questi noduli, tuttavia, non sono risultati maligni all’esame citologico e potrebbero essere di natura fibrosa. In conclusione, l’US-E sembra essere uno strumento di valore limitato nella diagnosi del tumore della tiroide nell’acromegalia.

  1. The effectiveness of external beam radiotherapy for acromegaly is not affected by previous pituitary ablative treatments

    Reed, P.I.; Joplin, G.F. (Department of Medicine, Royal Postgraduate Medical School, Hammersmith Hospital, London (UK)); Speirs, C.J. (Department of Clinical Pharmacology, Royal Postgraduate Medical School, Hammersmith Hospital, London (UK)); Morrison, R. (Department of Radiotherapy, Royal Postgraduate Medical School, Hammersmith Hospital, London (UK)); Aber, V. (Department of Medical Physics, Royal Postgraduate Medical School, Hammersmith Hospital, London (UK))

    1990-01-01

    Thirty-three acromegalic patients were treated with radiotherapy and followed up for at least 3 years (mean 6 years, range 3 to 12). Seventeen had not had previous pituitary ablative therapy and 16 had. The mean GH level for these two groups before radiotherapy was comparable at 98 and 119 mlU/l. The observed frequency of reaching <10 mlU/L was 53% and 75% of patients in the two groups, respectively, the mean observed falls in growth hormone level were 81 and 85% of the initial level, and the calculated exponential decline rate of GH level was 72 and 52% per fyear. Considering all 35 patients, requirement for pituitary hormone replacement therapy increased from 15 patients before radiotherapy to 20 after radiotherapy, being mostly those who had had prior ablative therapies. There were no complications attributable to the radiotherapy treatment. It appears that radiotherapy is equally efficacious whether a prior unsuccessful ablative procedure had been used or not. (author).

  2. 培维索孟治疗肢端肥大症%Pegvisomant treatment of acromegaly

    李乃适

    2008-01-01

    生长激素(Growth Hormone,GH)分泌过多,在骨骺尚未闭合时期不断促进长骨生长,使患者身高不断增长,称为巨人症;在骨骺已经闭合以后则促进扁骨等膜化骨增宽增厚,导致面容变形,手足增大,肢端变粗,称为肢端肥大症;如起病时骨骺尚未闭合而就诊时已闭合,则兼备二者的临床表现,称为肢端肥大性巨人症。无论诊断名称如何,其本质在于生长激素分泌过多,

  3. Using Growth Hormone Levels to Detect Macroadenoma in Patients with Acromegaly

    Ji Young Park

    2014-12-01

    Full Text Available BackgroundThe aim of this study was to assess the clinical differences between acromegalic patients with microadenoma and patients with macroadenoma, and to evaluate the predictive value of growth hormone (GH levels for early detection of macroadenoma.MethodsWe performed a retrospective analysis of 215 patients diagnosed with a GH-secreting pituitary adenoma. The patients were divided into two groups: the microadenoma group and the macroadenoma group, and the clinical parameters were compared between these two groups. The most sensitive and specific GH values for predicting macroadenoma were selected using receiver operating characteristic (ROC curves.ResultsCompared with the microadenoma group, the macroadenoma group had a significantly younger age, higher body mass index, higher prevalence of hyperprolactinemia and hypogonadism, and a lower proportion of positive suppression to octreotide. However, there were no significant differences in the gender or in the prevalence of diabetes between the two groups. The tumor diameter was positively correlated with all GH values during the oral glucose tolerance test (OGTT. All GH values were significantly higher in the macroadenoma group than the microadenoma group. Cut-off values for GH levels at 0, 30, 60, 90, and 120 minutes for optimal discrimination between macroadenoma and microadenoma were 5.6, 5.7, 6.3, 6.0, and 5.8 ng/mL, respectively. ROC curve analysis revealed that the GH value at 30 minutes had the highest area under the curve.ConclusionThe GH level of 5.7 ng/mL or higher at 30 minutes during OGTT could provide sufficient information to detect macroadenoma at the time of diagnosis.

  4. Homogeneity in the growth hormone-lowering effect of dopamine and somatostatin in acromegaly.

    Oppizzi, G; Botalla, L; Verde, G; Cozzi, R; Liuzzi, A; Chiodini, P G

    1980-09-01

    We have studied the effect of maximally inhibiting doses of dopamine (DA) or somatostatin on GH levels in 39 acromegalic patients. The GH-lowering effects of the two drugs were highly variable in different patients. A significant correlation (r = 0.45; P affect the response to somatostatin. We conclude that the GH-secreting cells of acromegalic patients contain separate receptors for DA and somatostatin. We hypothesize that the partial or total lack of responsiveness to DA or somatostatin may be due to the loss of receptors for these agents on the GH-secreting neoplastic cells.

  5. The growth hormone receptor polymorphism in patients with acromegaly: relationship to BMI and glucose metabolism.

    Turgut, Sebahat; Akın, Fulya; Ayada, Ceylan; Topsakal, Senay; Yerlikaya, Emrah; Turgut, Günfer

    2012-09-01

    The aim of this study was to investigate association between the frequencies of Growth Hormone receptor (d3GHR) gene polymorphisms and some clinical parameters of acromegalic patients. Total of 35 acromegalic patients were enrolled to study. The d3GHR polymorphism was identified by using polymerase chain reaction from peripheral blood samples. The levels of systolic and diastolic blood pressure, BMI, fasting plasma glucose (FPG), Fasting insulin, HOMA-IR, IGF-I, GH, IGFBP3, triglyceride, HDL and LDL cholesterol concentrations were evaluated. The frequencies of d3GHR genotypes were found as follows; 5 (14.3%) subjects had d3/d3, 11 (31.4%) had d3/fl and 19 (54.3%) had fl/fl in patients. The prevalence of the d3 and fl alleles was 30 and 70%, respectively. Systolic blood pressure, fasting insulin and HOMA-IR was found significantly increased in homozygote d3GHR genotype group compared to d3/fl subjects (P affect BMI, systolic blood pressure and insulin regulation. At the same time we can say homozygote d3GHR genotype and d3 allele carriers may have more risk than other genotypes for high BMI.

  6. Radiation optic neuropathy after external beam radiation therapy for acromegaly : report of two cases

    van den Bergh, ACM; Hoving, MA; Links, TP; Dullaart, RPF; Ranchor, AV; ter Weeme, CA; Canrinus, AA; Szabo, BG; Pott, JWR

    2003-01-01

    For diagnosing radiation optic neuropathy (RON) ophthalmological and imaging data were evaluated from 63 acromegalic patients, irradiated between 1967 and 1998. Two patients developed RON: one patient in one optic nerve 10 years and another patient in both optic nerves 5 months after radiation thera

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

    Sreenan, Seamus

    2012-01-01

    Asymptomatic pituitary abnormalities occur in about 10% of cranial magnetic resonance imaging scans, but metastatic carcinoma of the pituitary gland is rare: 133 cases have been reported. Two thirds secreted either prolactin or adrenocorticotropic hormone, and another 24% were non-secreting.

  8. The evaluation of QT intervals during diagnosis and after follow-up in acromegaly patients.

    Baser, Husniye; Akar Bayram, Nihal; Polat, Burcak; Evranos, Berna; Ersoy, Reyhan; Bozkurt, Engin; Cakir, Bekir

    2014-01-01

    Introdução: O estudo teve como objectivo a determinação do intervalo QT em doentes com acromegália e a análise da correlação entre o intervalo QT e a concentração de hormona do crescimento e de IGF-1 (insulin-like growth factor-1). Material e Métodos: O estudo englobou 41 doentes com acromegália. O grupo de controlo englobou 41 indivíduos com características semelhantes no que se refere a comorbilidades, idade e género. A electrocardiografia de doentes com acromegália foi obtida no início do estudo e após o follow-up. Foi apenas obtido um electrocardiograma no grupo de controlo. Foram calculados o QT máximo, QT mínimo, dispersão do intervalo QT, QT máximo corrigido, QT mínimo corrigido e dispersão do intervalo QT corrigido. Resultados: Os valores do QT máximo basal, dispersão do intervalo QT, QT máximo corrigido e dispersão do QT corrigido foram significativamente mais prolongados no grupo de doentes com acromegália do que nos controlos. O QT máximo corrigido e a dispersão do QT corrigido foram significativamente mais curtos durante o seguimento clínico, quando comparados com os valores basais dos doentes. O QT máximo, dispersão do QT, QT máximo corrigido e dispersão do QT corrigido durante o seguimento clínico não foram estatisticamente diferentes dos valores obtidos nos controlos. Com excepção de uma correlação negativa entre os valores da hormona do crescimento e a dispersão do QT corrigido em doentes durante o seguimento clínico, nenhuma outra correlação foi assinalada entre os valores do intervalo QT e as concentrações de hormona do crescimento e de IGF-1. Concluiu-se que a dispersão do intervalo QT está correlacionada com a duração da doença nos doentes com acromegália. Discussão: Em doentes com acromegália, é importante a detecção de preditores clínicos de arritmia cardíaca. A dispersão do intervalo QT é considerada um preditor relevante de arritmias ventriculares. Os doentes com acromegália englobados no nosso estudo apresentaram intervalos QT prolongados, quando comparados com os controlos. Conclusão: A avaliação do risco cardiovascular em doentes com acromegália deve englobar a determinação do intervalo QT.

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

    G.B. Camilo

    2015-01-01

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

  10. Long-term effects of radiotherapy for acromegaly on circulating prolactin

    Ciccarelli, E.; Corsello, S.M.; Besser, G.M.; Wass, J.A.H. (Department of Endocrinology, St. Bartholomew' s Hospital, West Smithfield, London (UK)); Plowman, P.N.; Jones, A.E. (Department of Radiotherapy, St. Bartholomew' s Hospital, West Smithfield, London (UK)); Touzel, R.; Rees, L.H. (Department of Chemical Endocrinology, St. Bartholomew' s Hospital, West Smithfield, London (UK))

    1989-01-01

    In 61 acromegalic patients, serum PRL was assessed (off medical treatment) before and 2 to 12 (mean 6 p) years after external beam radiotherapy. Before radiotherapy elevated PRL levels were present in 22 of 35 males (63%) and 12 of 26 females (46%) and were above 1000 mU/l in 11 males and 5 females. When studied for up to 5 years after radiotherapy, 22 or 23 (96%) patients who had not had surgery and who had normal PRL preradiotherapy showed an increased PRL level and this was also seen in 17 or 27 (63%) who had hyperprolactinaemic initially. In contrast, 10 of 27 patients (37%) who had elevated pre-radiotherapy levels (all greater than 1000 mU/l) had a reduction in PRL values after radiotherapy. In all 11 patients who underwent surgery before radiotherapy, an increase in PRL was seen after radiotherapy. In the 21 patients followed for 10--12 years, the peak PRL value occurred 1--6 years after radiotherapy. After this, a progressive reduction of PRL to normal was seen. Normal levels were reached 4 to 10 years after radiotherapy. No correlation was found between pretreatment PRL values and final GH values in the whole group, nor between changes in PRL and the development of Impaired ACTH or TSH secretion. Thus, different patterns of PRL behaviour suggest that radiotherapy treatment may either produce hyperprolactinemia from mild hypothalamic damage or ablate PRL secreting cells if they were present in the tumour before treatment. These changes do not predict final GH results or the development of hypopituitarism after radiotherapy. (author).

  11. Patología cardiovascular en la acromegalia Cardiovascular Disease in Acromegaly

    L Fiszlejder; PM Fiszlejder

    2012-01-01

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

  12. Long-term effect of /sup 90/Y pituitary implantation in acromegaly

    Jadresic, A.; Jimenez, L.E.; Joplin, G.F.

    1987-01-01

    This report examines the long-term trends in GH levels and pituitary function in a group of 38 acromegalic patients who were selected insofar as we were able to follow them up for more than 10 years after a single dose /sup 90/Y interstitial pituitary irradiation as the sole treatment. Mean serum GH had fallen from 106 to 24 mIU/l within 3-6 months and then slowly declined to 4 mIU/l after 10 years. GH levels of less than or equal to 5 mIU/l during a 50 g oral glucose tolerance test were obtained in 8% of patients at 3-6 months and in 18% at 1 year, the cumulative percentage increasing to 53% at 10, and 76% at 14 years. The percentage of patients requiring hormone replacement therapy rose from nil pre-implant to 16% by 3-6 months, and then slowly increased to 39% by 14 years. Serial coned radiographs of the pituitary fossa were available for 32 patients. By 10 years, 16 showed thickening of the dorsum sellae and/or reduction of at least one diameter by 3 mm. Concerning symptoms, all 29 patients whose GH level fell to less than or equal to 5 mIU/l showed improvements, 22 becoming asymptomatic. Seven patients with lesser falls in GH levels (from a mean of 193 to a mean of 15 mIU/l) all improved, one becoming asymptomatic. Two showed no variation. These results show that /sup 90/Y pituitary implants have a cumulative effect over the years in inducting remission and hypopituitarism in acromegalic patients, the early decline in GH levels being swifter than from other forms of irradiation.

  13. 肢端肥大症与高血压临床诊治%Acromegaly and Hypertension

    岑晶; 顾锋

    2009-01-01

    肢端肥大症是一种临床少见的内分泌疾病,可引起心血管、呼吸系统及代谢异常等并发症,其中高血压常见,导致患者病死率增加.其发病机制目前尚未达到共识,本文结合相关文献,综述肢大患者高血压发病率、危险性及其可能发病机制.肢大现代治疗目标主要是采用合适的方法使生化指标尽早达标,将病死率降低至正常人水平.针对肢大并发高血压,提出目前包括手术、放疗及药物的肿瘤本身治疗及高血压相关治疗.经治疗后部分患者血压下降,但控制生长激素(GH)与胰岛素样生长因子-1(IGF-1)至正常范围内非常重要.

  14. Chinese guidelines for acromegaly(draft)%中国肢端肥大症诊治规范(草案)

    中华医学会内分泌学分会; 中华医学会神经外科学分会

    2006-01-01

    前言 肢端肥大症(以下简称肢大)是一种起病隐匿的慢性进展性内分泌疾病,其主要特征是体内产生过量的生长激素(GH)。近30年来,我国在肢大的诊断和治疗上已取得了长足的进步,但也存在许多不容忽视的问题,此诊治规范旨在总结、吸取我国现有的肢大诊疗经验,提高我国医生对肢大的认识,倡导规范化的诊断和治疗管理模式。

  15. 肢端肥大症的显微手术治疗%Microsurgical treatment of pituitary adenoma with acromegaly

    惠国桢; 朱晓江; 凌伟华; 李向东; 王之敏; 周幽心; 鲍耀东; 杜子威

    2000-01-01

    目的提高肢端肥大症的治疗效果.方法回顾分析1984年到1997年收治的135例肢端肥大症,其中并有糖尿病23例,并有高血压14例,有肢端肥大性心肌病5例;除3例经额下入路外其余均经口鼻蝶窦入路显微手术切除肿瘤.结果全组术后1周内软组织肿胀均减轻乃至消失;128例进行GH复查的患者中GH下降至5μg/L以下者66例(51.6%);并有糖尿病者20例血糖恢复正常;5例并有肢端肥大性心肌病术后心脏全部恢复正常;但并有高血压的患者术后血压下降不明显.全组无死亡率.结论肢端肥大性心肌病及继发性糖尿病是肢端肥大症患者致死的主要原因,应严加关注,正确处理.

  16. The diagnosis and treatment of acromegaly%肢端肥大症的诊断与治疗

    徐春; 肖新华

    2004-01-01

    目的:总结肢端肥大症的临床特征以及并发症.讨论多种诊断试验的合理性,指出最精确可行的诊断试验.比较各种治疗方法的优点,其中包括一种新药--生长激素受体拮抗剂.肢端肥大症是一种影响多脏器功能的内分泌疾病,其死亡率是一般人群的2~4倍.生长激素(GH)水平增加是该病的特点,由于GH呈脉冲性分泌,故不是诊断该病最可靠的标准.IGF-I能准确反映GH的分泌情况,是惟一最可靠的诊断肢端肥大症的筛选试验.另外,GH的许多生物效应由IGF-I介导.肢端肥大症的治疗目标是控制有关生化指标,稳定或减小肿瘤的体积,维持或恢复垂体的功能.肢端肥大症的治疗方法有手术、放疗以及药物治疗.目前可行的药物治疗有生长抑素类似物和多巴胺激动剂.本文综述了这些治疗的优点和局限性.新药pegvisomant是一种GH受体拮抗剂,与靶细胞表面的GH受体结合,阻断GH信号的传导和GH的作用.与其他药物不同,pegvisomant的药效不依赖于受体在垂体肿瘤的表达.已证实,pegvisomant能有效将IGF-I水平降到正常,在接受pegvisomant治疗的患者中有超过97%的患者,其IGF-I浓度降低到正常水平并且症状和体征也得到明显改善.因此,pegvisomant是一种有希望的治疗肢端肥大症的新药,将为肢端肥大症的治疗带来发展.

  17. Advances in Medical Treatment for Acromegaly%肢端肥大症的药物治疗进展

    俞文华; 董小巧; 车志豪

    2006-01-01

    近年有关肢端肥大症的临床药物治疗取得了突破性进展,一些新药或改良衍生药物使绝大部分患者的临床症状和内分泌代谢异常得到了有效控制,成为临床治疗肢端肥大症的重要辅助手段,甚至在一定程度上取代了传统手术和放疗.本文就药物治疗肢端肥大症的进展作一综述.

  18. Effects of bromocriptine on CSF proteins and amines in patients with empty sella syndrome, acromegaly and prolactin producing pituitary adenomas.

    Brismar, K; Sidén, A; Werner, S

    1981-01-01

    The effect of the dopamine agonist bromocriptine (5-40 mg/day) on cerebrospinal fluid proteins and amines was studied in 7 hyperprolactinemic patients, 4 with empty sella syndrome and 3 patients with pituitary adenoma. Small as well as high doses of bromocriptine depressed the endogenously formed dopamine, noradrenalin and adrenalin. Five patients initially exhibited changes consistent with slight to marked blood-cerebrospinal-fluid (CSF) barrier disturbances and 5 abnormal CSF-protein fractions. One CSF-protein fraction (isolelectric points (pI) approximately 5.3 pH-units) became more prominent during bromocriptine treatment. Analyses of his fraction indicated that it represented a transferrin component. It is stated that bromocriptine treatment besides affecting amine and trace metal metabolism also affects protein metabolism.

  19. Acromegaly in a multiple endocrine neoplasia type 1 (MEN1) family with low penetrance of the disease

    Dreijerink, KMA; van Beek, AP; Lentjes, EGWM; Post, JG; van der Luijt, RB; Carnninga-van Dijk, MR; Lips, CJM

    2005-01-01

    Multiple endocrine neoplasia type 1 (MEN1) is an inherited syndrome that is characterised by the occurrence of tumours in the parathyroid glands, the endocrine pancreas, the pituitary gland and the adrenal glands and by neuroendocrine carcinoid tumours, often at a young age. The penetrance of MEN1 a

  20. Acromegaly Is More Severe in Patients With AHR or AIP Gene Variants Living in Highly Polluted Areas.

    Cannavo, S; Ragonese, M; Puglisi, S; Romeo, P D; Torre, M L; Alibrandi, A; Scaroni, C; Occhi, G; Ceccato, F; Regazzo, D; De Menis, E; Sartorato, P; Arnaldi, G; Trementino, L; Trimarchi, F; Ferrau, F

    2016-04-01

    In this multicentric study, we aimed to correlate the occurrence of AHR and/or AIP. genes variants in acromegalic patients with the disease severity and/or with the response to somatostatin analogs (SSa) treatment, according to pollution exposition.

  1. Severe scalp hair loss in a female patient with acromegaly treated with lanreotide autogel after unsuccessful surgery.

    Alvarez-Escola, Cristina; Cárdenas-Salas, Jersy J; Pelegrina, Beatriz; Sanz-Valtierra, Angel; Lecumberri, Beatriz

    2015-11-01

    Scalp hair loss is an underreported adverse event of somatostatin analogs therapy that in severe cases may require treatment withdrawal. It can be related to an acute decrease in GH/IGF-1 levels, but a direct effect cannot be ruled out.

  2. New progress in the medical treatment for acromegaly%肢端肥大症的内科治疗新进展

    顾锋

    2006-01-01

    @@ 1 简介 肢端肥大症(以下简称"肢大")是由于过度的生长激素(GH)分泌导致的一组疾病,其病因绝大多数是由于垂体GH分泌型肿瘤导致.传统的治疗方法通常是以手术作为首选治疗,对于残留肿瘤给予放射治疗.

  3. Clinical analysis of treatment of 79 cases with acromegaly%肢端肥大症患者79例临床治疗分析

    潘绵顺; 孙海辉; 王鹏; 汪业汉; 郭裕天; 黄润生

    2009-01-01

    目的 探讨使用长效生长抑素类似物结合神经外科切除或伽玛刀治疗后激素变化和影像学复查来评价肢端肥大症患者治疗疗效.方法 肢端肥大症患者79例,其中男性51例,女性28例,分为长效生长抑素(兰瑞肽lanreotideTM)结合神经外科切除治疗(A组)和长效生长抑素(兰瑞肽lanreotideTM)结合伽玛刀治疗组(B组),所有患者均给予肌注长效生长抑素,在药物使用结束4 W后对患者进行临床评估并施行经蝶窦手术切除肿瘤或者接受伽玛刀治疗.随访瘤体体积变化及内分泌水平变化.结果 多数患者的临床症状得到显著改善.与垂体微腺瘤组相比,药物治疗对垂体大腺瘤组体积缩小有更明显的作用.手术组微腺瘤组明显优于大腺瘤组;放射外科组结果:肿瘤缩小概率随随访时间的延长会明显提高,而且随着随访时间的延长,生长激素(GH)和胰岛素生长因子(IGF-I)值降至正常的概率明显提高.结论 生长抑素类似物(兰瑞肽lanreotideTM)结合神经外科切除或伽玛刀治疗肢端肥大症患者可以获得较好的临床疗效,远期疗效有待于进一步观察.

  4. Female gonadal function before and after treatment of acromegaly%女性肢端肥大症患者治疗前后的性腺功能

    李娜

    2011-01-01

    @@ 肢端肥大症为罕见疾病,发病隐匿,常至中年方获诊断,因而目前对该病女性患者中性腺功能障碍的发生率及原因所知甚少.完全或部分性促性腺激素缺乏可能与肿瘤占位效应(大腺瘤)及(或)高催乳素血症有关.

  5. Pegvisomant在肢端肥大症治疗中的地位%The place of pegvisomant in the management of acromegaly

    李乃适; 肖新华

    2004-01-01

    目的:阐明pegvisomant控制肢端肥大症及其并发症的机制.评价pegvisomant与其他药物治疗及非药物治疗在临床疗效方面的比较.复习pegvisomant对肢端肥大症特征性代谢改变所产生的效应,主要着重于糖耐量、皮质醇清除率和脂代谢.生长激素(GH)受体拮抗剂的发展是因为一小部分用传统疗法不能完全控制的肢端肥大症患者附加治疗的需要,同时也因为认识到控制GH/IGF-I轴在像乳腺癌和糖尿病微血管病变这样的疾病的治疗潜力.Pegvisomant的"设计"应用了在上一个10年我们对细胞因子及其受体认识上的飞速进展.GH是一个191肽,包括2个二硫键、4个α螺旋,分子量约22000d.人生长激素(hGH)第3个α螺旋的第120位的甘氨酸对于GH的生物活性是非常关键的;如果易之以其他氨基酸残基,则GH类似物即不再以激动剂的身份作用,而是作为生长的抑制剂.Pegvisomant是191肽GH的类似物,与第120位做氨基酸替换即产生拮抗剂,并做了额外的氨基酸替换,以保证位点1的结合.该拮抗剂被加入聚乙二醇进一步加以修饰,使其T1/2延长而免疫原性减弱.当GH/IGF-I轴的严密控制的需要已被令人信服地证实的时代来临时,而垂体手术和多巴胺激动剂、生长抑素类似物合并用药仍然留下为数甚多的患者不能得到充分控制,pegvisomant的应用对于肢端肥大症的现行治疗实在是一个受欢迎的附加举措.长期临床研究提示,pegvisomant在迄今为止的所有肢端肥大症的药物治疗中是最强的,40mg的剂量可使97%的患者血清IGF-I水平降至年龄相关的参考值范围.虽然pegvisomant治疗的长期效应在肢端肥大症的代谢改变和垂体瘤生长方面还需要更多资料,它仍然代表着多年来肢端肥大症药物的最激动人心的进展.

  6. 肢端肥大症继发糖尿病酮症酸中毒临床分析%Clinical Analysis of Acromegaly Complicatiag Diabetic ketoacidosis

    丁奇龙

    2005-01-01

    糖代谢紊乱是肢端肥大症的一个常见的临床特征。由于垂体分泌过量的生长激素(GH)可造成显著的胰岛素抵抗,继而引发糖代谢紊乱。北京协和医院报道的一组肢端肥大症550例,做葡萄糖耐量试验(OGTT),糖尿病的发生率为18.5%,糖耐量异常(IGT)为40%。Arya等资料中56%的肢端肥大症继发糖尿病。但由肢端肥大症引起的糖尿病酮症酸中毒(DKA)则罕见,国外仅有个案报道。由于肢端肥大症起病隐匿、进展缓慢,因而常常不能及时就诊明确诊断,有时以DKA为首发表现,更易造成误诊。现将我们收治的4例患者,其中3例以DKA为首发表现,现报道如下。

  7. 60例肢端肥大症患者心脏结构和功能改变%An Echocardiographic Study of Sixty Patients with Acromegaly

    李群; 王捷; 徐秀英; 李继红

    2006-01-01

    目的通过超声心动图检查,观察肢端肥大症患者心脏结构和功能的改变.方法选择60例肢端肥大症患者为实验组.选择34例性别和年龄相仿的健康者作为对照组.以上2组均行超声心动图检查,并做结果分析.将实验组中17例合并糖尿病和糖耐量减低者定为亚组病例,并进行亚组分析.结果1)实验组左心室舒张末期内径(LVIDd)、室间隔厚度(IVST)、左心室后壁厚度,心肌质量(LVM)及心肌质量指数(LVMi)较对照组明显增加,LVMi达到左心室肥厚(LVH)的比例为46.7%.经多元逐步回归分析,病程和血清生长激素(GH)浓度为心肌肥厚的主要影响因素.2)实验组射血分数(EF)和左心室短轴缩短率(FS)虽较对照组低,但仍在正常范围.3)实验组左心室等容舒张时间(IRT)和二尖瓣减速时间(MDT)较对照组明显延长.4)实验组中继发糖尿病/糖耐量减低(DM/IGT)组与非DM/IGT组进行比较,前者仅室间隔(IVST)有较明显增厚,其余各项参数2组间差异均无统计学意义.结论肢端肥大症患者心脏结构的改变,以心肌肥厚、心肌质量增加和心腔扩大为主.心脏功能的改变以舒张功能损害为主,而心脏收缩功能则可在较长时间内维持正常.

  8. 无垂体瘤影像学证据的肢端肥大症%Acromegaly without imaging evidence of pituitary adenoma

    杨晓平

    2011-01-01

    @@ 肢端肥大症为生长激素(GH)及胰岛素样生长因子Ⅰ(IGF-Ⅰ)异常升高所致,发病率3~4/1 000 000.临床表现为特殊面貌和肢端肥大,可并发风湿性疾病、心血管疾病、呼吸系统疾病以及代谢性疾病.诊断主要依据为口服葡萄糖耐量试验中服糖后GH最低值大于1 ng/ml以及按年龄、性别相应的IGF-Ⅰ水平升高.

  9. 培维索孟治疗肢端肥大症疗效的系统评价%Curative effect of pegvisomant on acromegaly: a systematic review

    罗谦; 姜曙; 周培志; 蔡博文

    2015-01-01

    目的 评价培维索孟治疗肢端肥大症的疗效和安全性.方法 系统性检索Cochrane Library、Medline、Embase、中国知网、维普等数据库,对培维索孟治疗肢端肥大症的疗效和安全性进行描述性的系统评价.结果 ①培维索孟能缓解患者部分症状;②培维索孟能使患者血清胰岛素生长因子-l (IGF-1)明显降低,同时生长激素可能升高;③培维索孟不影响肿瘤大小;④培维索孟短期不良反应较少.结论 培维索孟能改善肢端肥大症患者的症状,有效降低血清IGF-1水平,短期使用有较好的安全性.

  10. 肢端肥大症合并肠多发性息肉病1例%Acromegaly Complicated with Multiple Intestinal Polyps:A Case Report

    林晓琳; 齐晓光; 孙罡; 程时丹; 王立夫

    2009-01-01

    @@ 病例:患者男,69岁.因"肋骨渐突出20年,面容渐变10年伴间歇性大便带血1年余"于2008年10月15日入院.患者20年前无诱因下出现两侧肋缘肋骨逐渐突出,10年前始发现面容逐渐改变,双手、双足肢端粗大,舌体肥厚,下唇外翻.声音变粗.1年前无明显诱因下出现便血,量少,伴有黏液,色鲜红,血与粪便不相混合,便血可自行缓解.

  11. Combination of Acromegaly and Klinefelter syndrome: a case report%肢端肥大症合并Klinefelter综合征1例报告

    徐剑; 方红娟; 王芳; 钟历勇

    2016-01-01

    目的 总结肢端肥大症合并Klinefelter综合征患者的诊治经验.方法 对1例肢端肥大症术后性腺功能减退的患者进行临床分析及鉴别诊断,复习相关文献,明确患者垂体生长激素瘤术后的治疗策略,对其性腺功能减退进行定位诊断,进而明确病因.结果 患者垂体瘤术后GH及IGF-Ⅰ水平不达标,给予生长抑素治疗,根据其为高促性腺激素型性腺功能减退,染色体核型分析为47,XXY,确诊其为Klinefelter综合征.结论 反馈调节是内分泌系统的基本特征,对腺体功能评价,必须遵循功能-定位-病因的诊断程序,才能准确定位,明确病因.

  12. 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.

    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.

  13. Somatostatin analogues do not affect calcium metabolism in patients with acromegaly and primary hyperparathyroidism [corrected] due to MEN 1-like syndrome.

    Bogazzi, F; Lombardi, M; Russo, D; Sardella, C; Raggi, F; Brogioni, S; Cetani, F; Ceccarelli, C; Mariani, G; Basolo, F; Martino, E

    2011-02-01

    Patients with clinical features of MEN 1 without mutations in the menin gene fulfill the criteria of MEN1-like syndrome. Primary hyperparathyroidism (PHP) is the most frequent clinical finding in both syndromes and is usually treated by surgery. However, PHP has been reported to respond to somatostatin analogues (SSA) in MEN 1 patients. 7 patients with PHP in the context of MEN 1-like syndrome (and absence of mutations in the menin gene) were enrolled in the study and treated with SSA for 6 months for the non-PHP disease before parathyroidectomy. Serum ionized calcium, phosphorus, and PTH concentrations, and 24-h urinary calcium and phosphorus excretion were measured before and after SSA therapy. Mean serum ionized calcium, phosphorus, and PTH concentrations did not significantly change after a 6-month course with SSA. SSA scintigraphy did not reveal uptake in the neck region corresponding to the parathyroid adenoma identified at surgery and confirmed at histology. However, immunohistochemistry revealed SS-type 2A receptor in parathyroid tissue samples of 6 out of 7 patients. SSA therapy does not affect calcium-phosphorus metabolism in patients with MEN 1-like syndrome, suggesting that the drug has no role in controlling PHP in these subset of patients.

  14. Immunoreactivity score using an anti-sst2A receptor monoclonal antibody strongly predicts the biochemical response to adjuvant treatment with somatostatin analogs in acromegaly

    F. Gatto (Federico); R.A. Feelders (Richard); R. van der Pas (Rob); J.M. Kros (Johan); M. Waaijers (Marlijn); D. Sprij-Mooij (Diana); S.J.C.M.M. Neggers (Bas); A. van der Lelij (Allegonda); F. Minuto (Francesco); S.W.J. Lamberts (Steven); W.W. de Herder (Wouter); D. Ferone (Diego); L.J. Hofland (Leo)

    2013-01-01

    textabstractContext: Somatostatin receptor subtype 2 (sst2A) protein expression has been demonstrated to positively correlate with somatostatin analog treatment outcome in GH-secreting adenomas. Recently, a new rabbit monoclonal anti-sst2A antibody (clone UMB-1) has been validated as a reliable meth

  15. Italian Society for the Study of Diabetes (SID)/Italian Endocrinological Society (SIE) guidelines on the treatment of hyperglycemia in Cushing's syndrome and acromegaly.

    Baroni, M G; Giorgino, F; Pezzino, V; Scaroni, C; Avogaro, A

    2016-02-01

    Hyperglycemia is a common feature associated with states of increased growth hormone secretion and glucocorticoid levels. The purpose of these guidelines is to assist clinicians and other health care providers to take evidence-based therapeutic decisions for the treatment of hyperglycemia in patients with growth hormone and corticosteroid excess. Both the SID and SIE appointed members to represent each society and to collaborate in Guidelines writing. Members were chosen for their specific knowledge in the field. Each member agreed to produce-and regularly update-conflicts of interest. The authors of these guidelines prepared their contributions following the recommendations for the development of Guidelines, using the standard classes of recommendation shown below. All members of the writing committee provided editing and systematic review of each part of the manuscript, and discussed the grading of evidence. Consensus was guided by a systematic review of all available trials and by interactive discussions.

  16. Truncated somatostatin receptor 5 may modulate therapy response to somatostatin analogues--Observations in two patients with acromegaly and severe headache

    Marina, Djordje; Burman, Pia; Klose, Marianne;

    2015-01-01

    of sst2a in 50-75% and sst5 in 75-100% of tumor cells. The truncated sst5 variant TMD4 (sst5TMD4) showed expression in 20-57% of tumor cells. CONCLUSIONS: A poor biochemical response to octreotide may be associated with tumor expression of a truncated sst5 variant, despite abundant sst2a expression...

  17. 病気の顔貌と容貌 (1) : 先端肥大症(Acromegaly)について

    佐々木, 悠; 二田, 哲博; 福島, 武雄; 川崎, 晃一; 奥村, 恂

    1990-01-01

    Acromegaly is the clinical condition which results from prolonged, excessive circulating levels of growth hormone (GH) in adults, which occurs in the young before epiphyseal fusion, is called pituitary giantism. Acromegaly was first described in 1886 by Pierre Marie, who noted “a striking non-congenital hypertrophy of the extremities". Benign pituitary tumors are by far the most common cause of acromegaly. Whether the tumor results from a hypothalamic disturbance or a primary pituitary tumor ...

  18. Les adénomes somatotropes: etude clinique, biologique et neuroradiologique portant sur 85 cas d'acromégalie suivis à l'Hôpital de la Timone.

    VALDES SOCIN, Hernan Gonzalo

    1999-01-01

    Introduction Selective acromegaly for acromegaly may have a wide range of remission rates, ranging from 40 to 80% in published series. These heterogeneous results rely not only on the surgeon experience, but also depend on the use of different biochemical remission criteria. Aim of the work The first part of this study is to evaluate clinical, biochemical and radiological data in a consecutive series of 85 patients diagnosed with acromegaly and followed-up by the endocrine team of...

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

    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

  20. Sweat secretion rates in growth hormone disorders

    Sneppen, S B; Main, K M; Juul, A

    2000-01-01

    While increased sweating is a prominent symptom in patients with active acromegaly, reduced sweating is gaining status as part of the growth hormone deficiency (GHD) syndrome.......While increased sweating is a prominent symptom in patients with active acromegaly, reduced sweating is gaining status as part of the growth hormone deficiency (GHD) syndrome....

  1. GROWTH HORMONE-, ALPHA-SUBUNIT AND THYROTROPIN-COSECRETING PITUITARY-ADENOMA IN FAMILIAL SETTING OF PITUITARY-TUMOR

    LINKS, TP; MONKELBAAN, JF; DULLAART, RPF; VANHAEFTEN, TW

    1993-01-01

    A patient with acromegaly and hyperthyroidism due to a growth hormone-, thyrotrophin- and alpha-subunit-secreting pituitary adenoma is described. His deceased father had suffered from a pituitary tumour, and was likely to have had acromegaly as well. Plasma growth hormone and insulin-like growth fac

  2. Anesthetic management and complications in pituitary adenoma patients with acromegaly%合并肢端肥大症的垂体腺瘤患者麻醉管理及并发症

    计根林; 施宇翔; 黄怡; 熊利泽; 谢莉; 顾建文; 张英民; 董辉

    2002-01-01

    目的探讨合并肢端肥大症对垂体腺瘤合并肢端肥大症患者的麻醉管理及并发症的发生是否有影响. 方法选在全麻下行经蝶窦或开颅垂体腺瘤切除术合并有肢端肥大症患者120例,另选36例年龄、体质量和性别相似的非肢端肥大症同类手术患者行比较研究,探讨合并肢端肥大症对麻醉诱导时面罩通气、气管插管困难和危险性,血流动力学及其他麻醉并发症的影响. 结果合并肢端肥大症患者面罩通气泄漏率,舌根肥大,上呼吸道梗阻,血氧饱和度(SpO2)≤90%发生率,困难插管评分(IDS)均明显高于对照组( P <0.01),糖尿病、高血压、心肌缺血的发病率以及血压(BP)波动幅度均高于对照组( P <0.01),血糖明显升高,pH值降低. 芬太尼、异丙酚、尼卡地平、艾司洛尔的用量亦明显大于对照组( P <0.05),心率(HR)改变,万可松用量,术中液体入量,FiO2,PaO2以及PaCO2均相似. 尿量较对照组为少( P <0.05). 结论合并肢端肥大症患者易发生呼吸道梗阻,面罩通气和气管插管困难,有显著的血流动力学改变,血糖、血压明显升高以及水钠潴留可导致麻醉并发症及危险性增加.

  3. Microsurgery for acromegaly: results of long-term follow-up%显微手术治疗肢端肥大症的长期随访分析

    凌伟华; 惠国桢; 李向东; 吴思荣; 徐峰; 祁震宇

    2008-01-01

    目的 评价经蝶窦显微外科手术治疗垂体生长激素(GH)腺瘤的长期疗效.方法 回顾分析176例经显微手术及经病理证实的垂体GH腺瘤的临床治疗和长期随访结果.结果 176例患者中,微腺瘤13例,小腺瘤45例,大腺瘤62例,巨腺瘤56例,除7例因故经颅手术,其余均经蝶窦手术.肿瘤全切除90例,近全切除49例,大部切除37例.随访平均11.9年,全组治愈率38.99%,长期缓解率37.10%,复发率3.98%.结论 显微手术是垂体GH腺瘤的首选治疗方法.术前GH水平、肿瘤的大小及术中肿瘤切除程度足影响长期疗效的重要因素;术后GH水平可预测患者的预后.

  4. 垂体瘤术后未获痊愈的肢端肥大症患者的临诊应对%Approach to the patient with persistent acromegaly after pituitary surgery

    张婷婷

    2011-01-01

    @@ 肢端肥大症是一种慢性病,起病隐匿,症状和体征表现多样,可导致多系统合并症.对垂体瘤术后未能痊愈的肢端肥大症患者应进行全面评估,包括生化检查,测定血清胰岛素样生长因子I(IGF-I)和葡萄糖抑制后的生长激素(GH)水平;影像学检查,确定残余瘤位置及其占位效应;体格检查,有无骨骼和软组织过度生长以及与肢端肥大症相关的体征;并全面检查各种合并症.

  5. Meta-analysis of the Effect of Octreotide in the Treatment of Acromegaly%奥曲肽治疗肢端肥大症疗效及安全性的Meta分析

    李珍珠; 马立新; 张文生; 李泽福

    2013-01-01

    目的:系统评价奥曲肽治疗肢端肥大症的疗效.方法:计算机检索Cochrane图书馆、Medline、Embase、中国生物医学文献数据库(CBM)、中国学术期刊全文数据库(CNKI)、万方数据库,根据Cochrane系统评价方法对纳入的随机对照实验(RCT)进行方法学评价,并应用Revman 5.0软件进行Meta分析.结果:共纳入5个RCT,共计218例患者.Jadad评分均>3分.Meta分析结果显示:实验组干预后的血清GH及IGF-1的浓度低于对照组[MD=-19.15,95% CI(-24.32,-13.98)]及[MD=-21.37,95%CI(-26.15,-16.58)],实验组肿瘤体积缩小率大于对照组[RR =24.25,95% CI(4.87,120.79)],实验组腹泻的发生率与对照组的差异无统计意义[RR=1.69,95%CI(0.78,3.64)].结论:奥曲肽能安全有效的降低肢端肥大症患者血清GH和IGF-1的浓度,并有利于缩小肿瘤体积.由于纳入试验质量和病例数的限制,该结论需进一步的研究证实.

  6. 12例垂体腺瘤并肢端肥大症的手术治疗体会%The experience of operation for the pituitary adenoma presenting with acromegaly in 12 cases

    唐忠; 朱炎昌

    2003-01-01

    @@ 自1996年1月至2001年5月,我院经鼻蝶窦显微外科治疗垂体腺瘤性肢端肥大症25例,其中12例存在鼻部骨性解剖变异,现就术中所见解剖变异及处理体会报道如下.

  7. Comparison and analysis of the international and domestic medical guidelines for the diagnosis and treatment of acromegaly%国际与国内肢端肥大症指南对比分析

    彭璐; 崔佳; 窦京涛

    2016-01-01

    肢端肥大症系一种由生长激素分泌异常增多而导致的较为少见的慢性内分泌系统疾病,临床上最主要的原因是生长激素(GH)瘤.该病起病隐匿,进展缓慢,可影响机体多个组织器官,患者的总体死亡率约为普通人群2倍,而平均寿命约减少10年.目前,为了提高肢端肥大症的诊治水平,中华医学会内分泌学分会(CSE)、美国临床内分泌医师协会(AACE)、美国内分泌协会(TES)和欧洲内分泌协会(ESE)分别发布临床诊疗指南.通过研读与对比三个指南在疾病的定位与定性诊断、并发症的评估、治疗方案的选择、治疗目标的控制和巨人症及肢端肥大症合并妊娠相关诊疗等方面存在的异同,文章进行详尽解读,以期更好地规范本病的诊疗流程.

  8. Evaluation of left ventricular functions by tissue Doppler imaging technique in patients with acromegaly%组织多普勒成像技术评价肢端肥大症患者左室功能

    江翠华; 汤燕芳; 周宁明

    2012-01-01

    目的 探讨组织多普勒成像技术(TDI)评价肢端肥大症患者左心室收缩及舒张功能的价值. 方法 通过TDI研究62例肢端肥大症患者和30例对照者左室收缩及舒张功能,进行参数比较. 结果 肢端肥大症组左室收缩功能指标收缩期峰值速度(Sm)与对照组相比,差异有统计学意义(P<0.05);左室舒张功能指标与对照组相比,舒张早期峰值速度(Em)、舒张晚期峰值速度(Am)、Em/Am的差异均有统计学意义(P<0.05). 结论 肢端肥大症患者存在左室收缩及舒张功能障碍,TDI可定量分析其左室收缩及舒张功能,为早期发现左室功能障碍提供客观依据.

  9. The changes of body composition in patients with acromegaly after pituitary adenoma surgery%肢端肥大症患者垂体生长激素瘤切除术后身体组分的改变

    吴勤勇; 朱惠娟; 顾锋; 潘慧; 邓洁英; 史轶蘩

    2010-01-01

    目的 探讨肢端肥大症患者病情活动期与缓解后身体组分的变化与生长激素(GH)、类胰岛素生长因子-1(IGF-1)水平的相关性.方法 治疗前后患者做生长激素葡萄糖抑制试验(OGTT),测定GH谷值、窄腹IGF-1水平;使用体脂分析仪测定身高、体质量、脂肪了丁分含量(fat%)、去脂肪量(FFM)和身体总水量(TBW).结果 活动期男性患者体重指数(BMI)和FFM显著高于正常对照,脂肪百分含量则显著低于后者;缓解后,患者GH谷值和IGF-1水平明显下降,BMI仍显著高于正常男性,而脂肪百分含量和FFM分别呈现增加和减少的趋势,与GH谷值和IGF1水平的降低显著相关.活动期女性患者与正常女性比较,BMI、FFM显著高于后者,而两者脂肪百分含量无显著差异;缓解后,患者FFM逐渐减少,与GH谷值显著相关,脂肪百分含量无明显变化.结论 肢端肥大症患者身体组分与正常人相比差异显著.缓解后身体组分发生变化,与GH谷值、IGF-1水平密切相关.

  10. 肢端肥大患者应用Bonfils纤维喉镜行气管插管的临床评价%Clinical evaluation of bronchial intubation of Bonfils intubation fiberscope in acromegaly patients

    易杰; 罗爱伦

    2006-01-01

    目的 评价对垂体瘤肢端肥大并有插管困难的患者应用Bonfils纤维喉镜行气管插管的方法.方法 经全麻气管插管行垂体瘤切除术的患者15例,术前先行气道评估,若Mallampati评分≥3级或甲颏距离<6 cm、张口度<3.5 cm,三项有一项者被选入.常规麻醉诱导后用Bonfils纤维喉镜插管,记录诱导前、插管前、插管后的血流动力学变化.按插入口咽腔、看见会厌、进入声门和置入气管导管的难易度评估插管情况并记录插管时间和失败率,同时随访插管后咽痛声嘶等不良反应.结果 插管后收缩压和心率均有增加(P<0.05).所有患者均一次插管成功,平均插管时间为(65.3±10.6)s,有4例(26.7%)患者看见会厌的难易度为一般,2例(13.3%)患者进入声门较困难.除2例患者外所有患者术后无咽痛、声嘶等不良反应.结论 Bonfils纤维喉镜对于插管困难的患者是一种简便、易行的插管装置.

  11. Analysis of factors related to diabetes secondary to pituitary adenoma with acromegaly%肢端肥大症的垂体腺瘤继发糖尿病的相关因素分析

    孔令胜; 姚维成; 栗世方; 贺昭忠; 成磊

    2015-01-01

    目的 探讨肢端肥大症的垂体腺瘤病人继发糖尿病的相关因素.方法 回顾性分析继发与未继发糖尿病的84例肢端肥大症垂体腺瘤病人在发病年龄、性别、时间、瘤体大小、术前血生长激素(GH)及激素免疫组化方面的不同,采用二分类多因素Logistic回归分析肢端肥大症垂体腺瘤病人继发糖尿病的相关因素.结果 与未继发糖尿病比较,继发糖尿病的肢端肥大症垂体腺瘤病人的发病时间更短,术前血GH更高,促甲状腺激素(TSH)免疫反应阳性率更高(P<0.05).Logistic回归分析统计结果显示:发病时间、术前血GH水平及TSH免疫阳性率的Exp (B)值分别为0.212、1.160、93.392 (P <0.05).结论 发病时间、术前血GH及TSH免疫阳性是肢端肥大症垂体腺瘤继发糖尿病的相关因素,TSH免疫阳性是主要因素;肿瘤分泌的TSH可能参与肢端肥大症垂体腺瘤继发糖尿病的病理生理过程.

  12. Diminished and irregular thyrotropin secretion with preserved diurnal rhythm in patients with active acromegaly%活动期肢端肥大症患者TSH分泌减少且不规则但仍有昼夜节律

    陈适; 曾正陪

    2009-01-01

    @@ 肢端肥大症患者常合并甲状腺肿,其大小与生长激素分泌过量的时间长短有关.有些可演变为有自主分泌功能的结节性甲状腺肿,但显性甲状腺功能亢进则罕见.

  13. β-Arrestin 1 and 2 and G protein-coupled receptor kinase 2 expression in pituitary adenomas: role in the regulation of response to somatostatin analogue treatment in patients with acromegaly.

    Gatto, Federico; Feelders, Richard; van der Pas, Rob; Kros, Johan M; Dogan, Fadime; van Koetsveld, Peter M; van der Lelij, Aart-Jan; Neggers, Sebastian J C M M; Minuto, Francesco; de Herder, Wouter; Lamberts, Steven W J; Ferone, Diego; Hofland, Leo J

    2013-12-01

    Recent in vitro studies highlighted G protein-coupled receptor kinase (GRK)2 and β-arrestins as important players in driving somatostatin receptor (SSTR) desensitization and trafficking. Our aim was to characterize GRK2 and β-arrestins expression in different pituitary adenomas and to investigate their potential role in the response to somatostatin analog (SSA) treatment in GH-secreting adenomas (GHomas). We evaluated mRNA expression of multiple SSTRs, GRK2, β-arrestin 1, and β-arrestin 2 in 41 pituitary adenomas (31 GHomas, 6 nonfunctioning [NFPAs], and 4 prolactinomas [PRLomas]). Within the GHomas group, mRNA data were correlated with the in vivo response to an acute octreotide test and with the GH-lowering effect of SSA in cultured primary cells. β-Arrestin 1 expression was low in all 3 adenoma histotypes. However, its expression was significantly lower in GHomas and PRLomas, compared with NFPAs (P affect GRK2 and β-arrestin expression in GHomas or in cultured rat pituitary tumor GH3 cells. Noteworthy, β-arrestin 1 was significantly lower (P < .05) in tumors responsive to octreotide treatment in vitro, whereas GRK2 and SSTR subtype 2 were significantly higher (P < .05). Likewise, β-arrestin 1 levels were inversely correlated with the in vivo response to acute octreotide test (P = .001), whereas GRK2 and SSTR subtype 2 expression were positively correlated (P < .05). In conclusion, for the first time, we characterized GRK2, β-arrestin 1, and β-arrestin 2 expression in a representative number of pituitary adenomas. β-Arrestin 1 and GRK2 seem to have a role in modulating GH secretion during SSA treatment.

  14. Perioperative nursing of two patients with acromegaly and McCune-Albright syndrome%2例McCune-Albright综合征合并生长激素腺瘤患者的围手术期护理

    谢文雅; 霍晓鹏; 张毅; 王娟

    2009-01-01

    总结了2例McCune-Albright综合征合并生长激素腺瘤患者行经蝶垂体腺瘤切除术的护理方法.患者均行神经导航辅助下经蝶窦入路垂体腺瘤切除术.术前重视患者的心理变化.为手术做好动态MRI等相关检查及治疗.术后严密观察患者有无继发性蝶鞍区出血、脑脊液漏、水电解质紊乱、垂体功能低下等并发症.患者手术顺利,术后恢复良好.患者术后生长激素水平均有明显下降,这内分泌学治愈.

  15. Cardiac and metabolic effects of chronic growth hormone and insulin-like growth factor I excess in young adults with pituitary gigantism.

    Bondanelli, Marta; Bonadonna, Stefania; Ambrosio, Maria Rosaria; Doga, Mauro; Gola, Monica; Onofri, Alessandro; Zatelli, Maria Chiara; Giustina, Andrea; degli Uberti, Ettore C

    2005-09-01

    Chronic growth hormone (GH)/insulin-like growth factor I (IGF-I) excess is associated with considerable mortality in acromegaly, but no data are available in pituitary gigantism. The aim of the study was to evaluate the long-term effects of early exposure to GH and IGF-I excess on cardiovascular and metabolic parameters in adult patients with pituitary gigantism. Six adult male patients with newly diagnosed gigantism due to GH secreting pituitary adenoma were studied and compared with 6 age- and sex-matched patients with acromegaly and 10 healthy subjects. Morphologic and functional cardiac parameters were evaluated by Doppler echocardiography. Glucose metabolism was assessed by evaluating glucose tolerance and homeostasis model assessment index. Disease duration was significantly longer (Pgigantism than in patients with acromegaly, whereas GH and IGF-I concentrations were comparable. Left ventricular mass was increased both in patients with gigantism and in patients with acromegaly, as compared with controls. Left ventricular hypertrophy was detected in 2 of 6 of both patients with gigantism and patients with acromegaly, and isolated intraventricular septum thickening in 1 patient with gigantism. Inadequate diastolic filling (ratio between early and late transmitral flow velocitygigantism and 1 of 6 patients with acromegaly. Impaired glucose metabolism occurrence was higher in patients with acromegaly (66%) compared with patients with gigantism (16%). Concentrations of IGF-I were significantly (Pgigantism who have cardiac abnormalities than in those without cardiac abnormalities. In conclusion, our data suggest that GH/IGF-I excess in young adult patients is associated with morphologic and functional cardiac abnormalities that are similar in patients with gigantism and in patients with acromegaly, whereas occurrence of impaired glucose metabolism appears to be higher in patients with acromegaly, although patients with gigantism are exposed to GH excess for a

  16. [Hirsutism and multinodular goiter in a 40-year-old female watchmaker].

    Mai, M; Tönjes, A; Trantakis, C; Wittekind, C; Stumvoll, M; Führer, D

    2013-09-01

    This article presents the case of a female patient with acromegaly caused by ectopic production of growth hormone-releasing hormone (GHRH) secretion. In the presence of typical clinical features of acromegaly but a lack of evidence for a pituitary adenoma the results of somatostatin receptor scintigraphy were indicative of a typical carcinoid of the lungs as the cause of the ectopic secretion of GHRH and the stimulation of pituitary gland growth hormone secretion resulting in acromegaly. Finally, the patient underwent curative surgical treatment.

  17. N-terminal pro-B-type natriuretic peptide in patients with growth hormone disturbances

    Andreassen, Mikkel; Faber, Jens; Vestergaard, Henrik;

    2007-01-01

    Acromegaly is associated with hypertrophic cardiomyopathy, hypertension and subsequent congestive heart failure. Impairment of cardiac function has also been associated with growth hormone deficiency (GHD). B-type natriuretic peptides (BNPs) have emerged as strong diagnostic and prognostic risk...

  18. Pituitary tumors containing cholecystokinin

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

    1987-01-01

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

  19. Growth hormone test

    ... special test is done to confirm this diagnosis.) Hypopituitarism (low function of the pituitary gland.) Risks Veins ... Read More Acromegaly Gigantism Growth hormone deficiency - children Hypopituitarism Pituitary tumor Review Date 2/3/2016 Updated ...

  20. TBG - blood test

    ... TBG levels may be due to: Acute illness Acromegaly (disorder caused by too much growth hormone) Hyperthyroidism (overactive thyroid) Malnutrition Nephrotic syndrome (symptoms that show kidney damage is present) Stress from ...

  1. Results of endoscopic transsphenoidal pituitary surgery in 40 patients with a growth hormone-secreting macroadenoma

    Wagenmakers, M.A.; Netea-Maier, R.T.; Lindert, E.J. van; Pieters, G.F.F.M.; Grotenhuis, A.J.; Hermus, A.R.M.M.

    2011-01-01

    OBJECTIVE: Transsphenoidal pituitary surgery (TS) is the primary treatment of choice for patients with acromegaly. Macroadenomas (>1 cm) are more difficult to resect than microadenomas (remission rate +/- 50% compared to +/- 90%). Besides the conventional microscopic TS, the more recently introdu

  2. Pituitary diseases : long-term clinical consequences

    Klaauw, Agatha Apolonia van der

    2008-01-01

    This thesis describes various studies during the long-term follow-up of patients after treatment for pituitary diseases. The focus of this thesis is acromegaly, growth hormone deficiency, sleep and quality of life. Various aspects are described.

  3. Pituitary tumors containing cholecystokinin

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

    1987-01-01

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

  4. [My hands hurt so much!].

    Rüst, Christoph A; Rosemann, Thomas; Knechtle, Beat

    2014-11-26

    We present the case of a 48-year-old man complaining of pain in his hands. In a first step, a two-sided carpal tunnel syndrome was found, and in the further course of the disease an acromegaly. The combinations of carpal tunnel syndrome, impaired glucose tolerance and radiological findings in the area of the sella turcica were the clues for the diagnosis of acromegaly.

  5. Clinical comparative analysis on patients with secondary and no secondary diabetes of pituitary adenoma with acromegaly%继发与不继发糖尿病的肢端肥大症型垂体腺瘤患者的临床比较分析

    孔令胜; 姚维成; 栗世方; 贺昭忠; 成磊

    2014-01-01

    目的:比较分析继发与不继发糖尿病的肢端肥大症型垂体腺瘤患者的临床资料,初步探讨肢端肥大症垂体腺瘤继发糖尿病的可能病理免疫学机制。方法回顾性分析2008年1月至2013年6月济宁医学院附属医院神经外科采用单鼻孔直达蝶窦入路显微手术切除的84例肢端肥大症垂体腺瘤患者的临床资料,运用免疫化学发光法测定术前基础血生长激素( GH)水平,使用多田公式( xyz/2)计算MRI上肿瘤体积,采用免疫组织化学的方法检测肿瘤内分泌激素的表达,采用χ2或t检验比较继发与不继发糖尿病的肢端肥大症垂体腺瘤患者在发病年龄、性别、发病时间、瘤体大小、基础血GH水平以及肿瘤激素免疫反应等方面的差异。结果20例(24%)肢端肥大症垂体腺瘤患者继发糖尿病。继发和不继发糖尿病的肢端肥大症垂体腺瘤患者男女均可发病(χ2=1.944, P =0.163),发病年龄多集中在41~50岁( P=0.652),MRI显示瘤体大小均有微腺瘤、大腺瘤、巨大腺瘤,差异均无统计学意义( P=1.000);发病时间分别集中在1~5年(55%)、6~10年(45%),差异有统计学意义(P=0.004);术前基础血GH水平分别为(42.83±8.70)ng/ml、(38.91±36.46)ng/ml,差异有统计学意义(t=5.253,P=0.031)。继发糖尿病的肢端肥大症垂体腺瘤患者促甲状腺激素(TSH)免疫反应平均光密度值(0.545±0.023)明显高于不继发糖尿病者(0.408±0.019),差异有统计学意义(t=5.336,P=0.001);TSH免疫反应阳性率(70%)亦明显高于不继发糖尿病的肢端肥大症患者(14%),差异有统计学意义(χ2=23.971, P=0.000)。结论继发与不继发糖尿病的肢端肥大症垂体腺瘤患者在发病年龄、性别、瘤体大小方面无明显差别,但二者在发病时间、术前基础血GH水平及肿瘤病理免疫反应方面存在明显差别,肿瘤细胞分泌的TSH可能参与了肢端肥大症垂体腺瘤继发糖尿病的病理生理过程。%Objective To compare and analyze the clinical data of patients with acromegalic pituitary adenoma between secondary and no secondary diabetes , and discuss the possible pathogenesis of acromegalic pituitary adenoma with secondary diabetic .Methods A total of 84 patients with acromegalic pituitary adenoma resected via single-nostril transspheniodal approach in Affiliated Hospital of Jining Medical College from Jan.2008 to Jun.2013 were analyzed retrospectively.Preoperative serum growth hormone(GH) levels were determined by chemiluminescent immunoassay .The tumor volume was calculated by the Coniglobus formula ( xyz/2 ) .The expression of endocrine hormone was detected by immunohistochemistry . The difference of acromegalic pituitary adenoma between secondary and no secondary diabetes in the age , gender,time of incidence,tumor size,basic blood GH level and tumor immune response were compared by usingχ2 or t test.Results Twenty cases(24%)in acromegalic pituitary adenoma patients complicated with secondary diabetes .Secondary and no secondary diabetes patients occurred in both men and women (χ2 =1.944,P=0.163),the incidence age often concentrated in 41-50 old years(P=0.652),MRI showed that tumor size were microadenomas , macroadenomas and giantadenomas , there were no significant differences ( P=1.000 ) .The incidence time of acromegalic pituitary adenoma with secondary diabetes mostly concentrated in 1-5 years ( 55%) , no secondary diabetes were more concentrated in 6-10 years ( 45%) , statistical differences were noted ( P=0.004 ) .The basic blood GH level of acromegalic pituitary adenoma with secondary and no secondary diabetes were ( 42.83 ±8.70 ) ng/ml and ( 38.91 ±36.46 ) ng/ml respectively,there was statistically significant difference between them ( t =5.253, P =0.031 ).Thyroid stimulation hormone(TSH)immunoreactive intensity(0.545 ±0.023)of acromegalic pituitary adenoma with secondary diabetes was significantly higher than that of no secondary diabetes ( 0.408 ±0.019 ) , the difference was statistically significant ( t=5.336 ,P=0.001 ) .The percentage of secondary diabetes patients with positive TSH immunoreactivity(70%)was significantly more than that of no secondary diabetes (14%) (χ2 =23.971,P =0.000).Conclusions There are no significant differences between secondary and no secondary diabetes of acromegalic pituitary adenoma in age ,gender,tumor size,but statistical differences are noted in incidence time ,preoperative basic blood GH level and pathological immune response .TSH of tumor cell secretion may be involved in the pathological and physiological process of acromegalic pituitary adenoma with secondary diabetes .

  6. Continuous positive airway pressure in the treatment of patients with acromegaly and obstructive sleep apnea hypo-ventilation syndrome%持续气道正压通气治疗老年肢端肥大症合并阻塞性睡眠呼吸暂停低通气综合征病例报道及文献复习

    刘歆; 陆志强; 符翠萍; 吴旭; 张舒淇; 李善群

    2014-01-01

    目的 分析老年肢端肥大症合并阻塞性睡眠呼吸暂停低通气综合征(OSAHS)患者的临床特点、可能的发生机制,以及治疗方法,为临床提供理论依据和指导.方法 总结近期收治的4例老年性肢端肥大症合并OSAHS患者的临床资料,分析患者接受持续气道正压通气(CPAP)呼吸机治疗的临床特征. 结果 患者存在重度OSAHS合并夜间重度低氧血症,并患有高血压和糖尿病存在垂体内分泌变化.CPAP呼吸机对患者OSAHS治疗有益.结论 临床肢端肥大症并发OSAHS较常见,老年人更易合并严重的睡眠呼吸暂停,患者有必要行多导睡眠图监测.CPAP可使OSAHS患者的临床症状得到缓解和改善.

  7. Acromegaly with diabetes mellitus or glucose intolerance: influence of transsphenoidal pituitary adenomas microsurgery for 77 cases%肢端肥大症合并糖尿病或糖耐量减退经蝶窦手术的疗效

    上官军发; 马文斌; 王任直; 杨众; 李桂林; 高俊; 陶蔚

    2005-01-01

    目的总结经蝶窦垂体生长激素(GH)腺瘤切除术对肢端肥大症合并继发性糖尿病或糖耐量减退病人的疗效.方法回顾性分析77例采用经单鼻孔或口鼻蝶改良入路显微手术切除肿瘤治疗该类病人的临床资料.结果肿瘤全切除加次全切除率85.7%;内分泌治愈加缓解56例(72.7%);血糖达到糖尿病一般控制以上标准者67例(87.0%);术后死亡1例.结论经蝶窦手术切除肿瘤能够纠正肿瘤高GH分泌状态,可在术后1周左右使继发性糖尿病及糖耐量减退得到控制.应注意对病人的心功能进行评估与保护.

  8. Acromegalia: comparación de los niveles séricos de IGF-I por dos inmunoensayos y su correlación con la prueba de tolerancia oral a la glucosa Acromegaly: Comparison of serum IGF-1 levels measured by two immunoassays and their correlation with the Oral Glucose Tolerance Test

    L. Boero; M S. Mallea Gil; M. Manavela; G. Stalldecker; K. Danilowicz; Guitelman, M.; Alfieri, A.; M.C. Ballarino; A. Chervin; N. García Basavilbaso; M. Glerean; M.G. Loto; J.A. Nahmías; A.S. Rogozinski; M. Servidio

    2010-01-01

    Introducción: La determinación de IGF-I en suero o plasma es una herramienta esencial en el diagnóstico y seguimiento de la acromegalia. Sin embargo, se deben tener presentes algunos inconvenientes en su medición por diferentes inmunoensayos. Objetivos: Evaluar dos inmunoensayos para la determinación de IGF-I y su correlación con el nadir de GH en el TTOG en pacientes acromegalicos. Materiales y métodos: Se analizaron 37 pacientes acromegálicos, 20 mujeres y 17 hombres. IGF-I fue determinada ...

  9. Governor Pio Pico, the monster of California...no more: lessons in neuroendocrinology.

    Login, Ivan S; Login, Jessica

    2010-01-01

    We hypothesize that Pio Pico, the last Mexican Governor of California, had acromegaly between at least ages 43 to 57, from 1844 to 1858, before Pierre Marie published the clinical description of acromegaly in 1886. Pico's probable growth hormone-secreting pituitary tumor likely infarcted spontaneously after 1858. The tumor infarction resulted in burnt-out acromegaly and probably restored normal pituitary function. Pearce Bailey published the first account of pituitary tumor infarction only in 1898. Pico's undiagnosed, misunderstood, profoundly acromegalic appearance was widely misinterpreted, leading to pervasive, degrading, and highly prejudicial comments. This landmark case study in neuroendocrinology provides the opportunity to re-examine elements of 19th century California and American history.

  10. AHR Over-Expression in Papillary Thyroid Carcinoma: Clinical and Molecular Assessments in a Series of Italian Acromegalic Patients with a Long-Term Follow-Up

    Mian, Caterina; Ceccato, Filippo; Barollo, Susi; Watutantrige-Fernando, Sara; Albiger, Nora; Regazzo, Daniela; de Lazzari, Paola; Pennelli, Gianmaria; Rotondi, Sandra; Nacamulli, Davide; Pelizzo, Maria Rosa; Jaffrain-Rea, Marie-Lise; Grimaldi, Franco; Occhi, Gianluca; Scaroni, Carla

    2014-01-01

    Aim Acromegaly reportedly carries an increased risk of malignant and benign thyroid tumors, with a prevalence of thyroid cancer of around 3–7%. Germline mutations in the aryl-hydrocarbon receptor (AHR) interacting protein (AIP) have been identified in familial forms of acromegaly. The molecular and endocrine relationships between follicular thyroid growth and GH-secreting pituitary adenoma have yet to be fully established. Our aim was to study the prevalence of differentiated thyroid cancer (DTC) in acromegaly, focusing on the role of genetic events responsible for the onset of thyroid cancer. Methods Germline mutations in the AIP gene were assessed in all patients; BRAF and H-N-K RAS status was analyzed by direct sequencing in thyroid specimens, while immunohistochemistry was used to analyze the protein expression of AIP and AHR. A set of PTCs unrelated to acromegaly was also studied. Results 12 DTCs (10 papillary and 2 follicular carcinomas) were identified in a cohort of 113 acromegalic patients. No differences in GH/IGF-1 levels or disease activity emerged between patients with and without DTC, but the former were older and more often female. BRAF V600E was found in 70% of the papillary thyroid cancers; there were no RAS mutations. AIP protein expression was similar in neoplastic and normal cells, while AHR protein was expressed more in PTCs carrying BRAF mutations than in normal tissue, irrespective of acromegaly status. Conclusions The prevalence of DTC in acromegaly is around 11% and endocrinologists should bear this in mind, especially when examining elderly female patients with uninodular goiter. The DTC risk does not seem to correlate with GH/IGF-1 levels, while it may be associated with BRAF mutations and AHR over-expression. Genetic or epigenetic events probably play a part in promoting thyroid carcinoma. PMID:25019383

  11. AHR over-expression in papillary thyroid carcinoma: clinical and molecular assessments in a series of Italian acromegalic patients with a long-term follow-up.

    Caterina Mian

    Full Text Available Acromegaly reportedly carries an increased risk of malignant and benign thyroid tumors, with a prevalence of thyroid cancer of around 3-7%. Germline mutations in the aryl-hydrocarbon receptor (AHR interacting protein (AIP have been identified in familial forms of acromegaly. The molecular and endocrine relationships between follicular thyroid growth and GH-secreting pituitary adenoma have yet to be fully established. Our aim was to study the prevalence of differentiated thyroid cancer (DTC in acromegaly, focusing on the role of genetic events responsible for the onset of thyroid cancer.Germline mutations in the AIP gene were assessed in all patients; BRAF and H-N-K RAS status was analyzed by direct sequencing in thyroid specimens, while immunohistochemistry was used to analyze the protein expression of AIP and AHR. A set of PTCs unrelated to acromegaly was also studied.12 DTCs (10 papillary and 2 follicular carcinomas were identified in a cohort of 113 acromegalic patients. No differences in GH/IGF-1 levels or disease activity emerged between patients with and without DTC, but the former were older and more often female. BRAF V600E was found in 70% of the papillary thyroid cancers; there were no RAS mutations. AIP protein expression was similar in neoplastic and normal cells, while AHR protein was expressed more in PTCs carrying BRAF mutations than in normal tissue, irrespective of acromegaly status.The prevalence of DTC in acromegaly is around 11% and endocrinologists should bear this in mind, especially when examining elderly female patients with uninodular goiter. The DTC risk does not seem to correlate with GH/IGF-1 levels, while it may be associated with BRAF mutations and AHR over-expression. Genetic or epigenetic events probably play a part in promoting thyroid carcinoma.

  12. Comparing two acromegalic patients with respect to central corneal thickness, intraocular pressure, and tear insulin-like growth factor levels before and after treatment

    Kan Emrah

    2015-01-01

    Full Text Available The aim of the study was to compare the central corneal thickness (CCT, intraocular pressure (IOP, and tear insulin-like growth factor-1 (IGF-1 levels of 2 patients with acromegaly before and after the surgical treatment of the disease. CCTs, IOP levels, and tear IGF-1 values showed a decrease after the treatment in 2 patients. As we found higher CCT, IOP, and tear IGF-1 levels in the active phase of the disease in two acromegaly patients, detailed information about the activity of the disease may be important before the examination of these patients.

  13. Impact of Growth Hormone on Cystatin C

    Lisa Sze; René L. Bernays; Cornelia Zwimpfer; Peter Wiesli; Michael Brändle; Christoph Schmid

    2013-01-01

    Background: Cystatin C (CysC) is an alternative marker to creatinine for estimation of the glomerular filtration rate (GFR). Hormones such as thyroid hormones and glucocorticoids are known to have an impact on CysC. In this study, we examined the effect of growth hormone (GH) on CysC in patients with acromegaly undergoing transsphenoidal surgery. Methods: Creatinine, CysC, GH and insulin-like growth factor-1 (IGF-1) were determined in 24 patients with acromegaly before and following transsphe...

  14. Impact of Growth Hormone on Cystatin C

    Sze, Lisa; René L. Bernays; Zwimpfer, Cornelia; Wiesli, Peter; Brändle, Michael; Schmid, Christoph

    2013-01-01

    BACKGROUND: Cystatin C (CysC) is an alternative marker to creatinine for estimation of the glomerular filtration rate (GFR). Hormones such as thyroid hormones and glucocorticoids are known to have an impact on CysC. In this study, we examined the effect of growth hormone (GH) on CysC in patients with acromegaly undergoing transsphenoidal surgery. METHODS: Creatinine, CysC, GH and insulin-like growth factor-1 (IGF-1) were determined in 24 patients with acromegaly before and following transs...

  15. Growth hormone-releasing hormone (GRH)-producing pancreatic tumor with no evidence of multiple endocrine neoplasia type 1.

    Kawa, S; Ueno, T; Iijima, A; Midorikawa, T; Fujimori, Y; Tokoo, M; Oguchi, H; Kiyosawa, K; Imai, Y; Kaneko, G; Kuroda, T; Hashizume, K; Osamura, R Y; Katakami, H

    1997-07-01

    The characteristic features of a 48-year-old male presenting with isolated acromegaly caused by a GRH-producing pancreatic endocrine tumor bearing no relation to MEN1 was reported. The clinical features, laboratory findings, and sellar enlargement were improved after removal of the pancreatic tumor. The resected pancreatic tumor showed positive GRH immunoreactivity and contained abundant GRH mRNA. This tumor is extremely rare and to date only 10 cases have been reported. In the management of acromegaly, the measurement of GRH is recommended and the search for an ectopic source will prevent unnecessary and potentially ineffective pituitary surgery.

  16. Endoscopic Decompression for Optic Neuropathy in McCune-Albright Syndrome.

    Noh, Jung-Hoon; Kong, Doo-Sik; Seol, Ho Jun; Shin, Hyung Jin

    2014-09-01

    McCune-Albright syndrome (MAS) is characterized by a triad of poly/monostotic fibrous dysplasia, café-au-lait macules and hyperfunctioning endocrinopathies including human growth hormone excess. Acromegaly as a manifestation of endocrine hyperfunction with MAS is uncommon. Surgical excision may be challenging due to the associated severe fibrous dysplasia of the skull base. Through the endoscopic procedures, we treated a case of MAS presenting with compressive optic neuropathy due to fibrous dysplasia and acromegaly caused by growth hormone secreting pituitary adenoma. We reviewed the literature on GH excess in MAS to highlight its surgical and medical challenges.

  17. Growth hormone deficiency and hyperthermia during exercise

    Juul, A; Hjortskov, N; Jepsen, Leif

    1995-01-01

    Sweat secretion is often disturbed in patients with GH secretory disorders. Hyperhidrosis is a classic feature of acromegaly, and it has recently been shown that GH-deficient patients exhibit decreased sweating capacity after pilocarpine stimulation of the skin. Thus, patients with GH-deficiency ......Sweat secretion is often disturbed in patients with GH secretory disorders. Hyperhidrosis is a classic feature of acromegaly, and it has recently been shown that GH-deficient patients exhibit decreased sweating capacity after pilocarpine stimulation of the skin. Thus, patients with GH...

  18. Musculoskeletal disorders. When are they caused by hormone imbalance?

    Brick, J E; Brick, J F; Elnicki, D M

    1991-11-01

    Often, the source of a musculoskeletal problem can be traced to an endocrine disorder. For example, carpal tunnel syndrome is not uncommon in patients who are pregnant or have diabetes, hypothyroidism, or acromegaly. Joint problems and arthritis are other common findings in diabetes, pregnancy, and hyperparathyroidism. Muscle weakness or stiffness is seen in both hypothyroidism and hyperthyroidism, and muscle wasting is a characteristic of adrenocorticoid insufficiency. Bone disorders are common with glucocorticoid excess, acromegaly, and hyperparathyroidism. Some presentations are a classic picture of a specific endocrine condition and are readily recognized if the index of suspicion is appropriately high.

  19. Random Secretion of Growth Hormone in Humans

    Prank, Klaus; Kloppstech, Mirko; Nowlan, Steven J.; Sejnowski, Terrence J.; Brabant, Georg

    1996-08-01

    In normal humans, growth hormone (GH) is secreted from a gland located adjacent to the brain (pituitary) into the blood in distinct pulses, but in patients bearing a tumor within the pituitary (acromegaly) GH is excessively secreted in an irregular manner. It has been hypothesized that GH secretion in the diseased state becomes random. This hypothesis is supported by demonstrating that GH secretion in patients with acromegaly cannot be distinguished from a variety of linear stochastic processes based on the predictability of the fluctuations of GH concentration in the bloodstream.

  20. Hypophyseal disorders and others

    1992-01-01

    920641 External pituitary irradiation ther-apy for acromegaly. JIN Zimeng(金自孟),et al. Dept Endocrinol, PUMC Hosp, Beijing,100730. Chin J Intern Med 1992; 31(7): 417-420. One hundred and twenty-eight acromegalypatients were followed up for 1-23 years(mean5.9±5.0 years) after extenal pituitary irradia-

  1. Algorithmic complexity of growth hormone release in humans

    Prank, K.; Wagner, M.; Brabant, G. [Medical School Hannover (Germany)

    1996-12-31

    Most hormones are secreted in an pulsatile rather than in a constant manner. This temporal pattern of pulsatile hormone release plays an important role in the regulation of cellular function and structure. In healthy humans growth hormone (GH) secretion is characterized by distinct pulses whereas patients bearing a GH producing tumor accompanied with excessive secretion (acromegaly) exhibit a highly irregular pattern of GH release. It has been hypothesized that this highly disorderly pattern of GH release in acromegaly arises from random events in the GH-producing tumor under decreased normal control of GH secretion. Using a context-free grammar complexity measure (algorithmic complexity) in conjunction with random surrogate data sets we demonstrate that the temporal pattern of GH release in acromegaly is not significantly different from a variety of stochastic processes. In contrast, normal subjects clearly exhibit deterministic structure in their temporal patterns of GH secretion. Our results support the hypothesis that GH release in acromegaly is due to random events in the GH-producing tumorous cells which might become independent from hypothalamic regulation. 17 refs., 1 fig., 2 tabs.

  2. Effects of a 7-day continuous infusion of octreotide on circulating levels of growth factors and binding proteins in growth hormone (GH)-treated GH-deficient patients

    Laursen, Torben; Møller, Jens; Fisker, Sanne;

    1999-01-01

    Abstract In patients with acromegaly, clinical improvement has been reported after octreotide (OCT) treatment, even in cases of only a moderate suppression of growth hormone (GH) levels. In rats, OCT suppresses IGF-I mRNA expression and generation of serum and tissue IGF-I levels. A direct effect...

  3. The Insulin-Sensitive Side of SHIP2

    Stephane Schurmans

    2001-01-01

    Full Text Available A substantial and increasing proportion of death and disability in the EU (and elsewhere is attributable to diseases associated with insulin resistance (i.e., decreased insulin sensitivity. Beside type II diabetes, other diseases like obesity, hypertension, atherosclerosis, hyperlipidaemia, polycystic ovarian syndrome, and acromegaly are indeed associated with insulin resistance [1].

  4. The ratio between serum levels of insulin-like growth factor (IGF)-I and the IGF binding proteins (IGFBP-1, 2 and 3) decreases with age in healthy adults and is increased in acromegalic patients

    Juul, A; Main, K; Blum, W F;

    1994-01-01

    administration. Serum levels of IGF-I and IGFBP-3 decrease with age in normal adults and are elevated in active acromegaly due to excessive GH secretion. However, the individual ratios between serum levels of IGF-I and IGFBP-3 in acromegalic and healthy adults have not been described previously....

  5. Fat deposition in the cavernous sinus in Cushing disease

    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.

  6. Clinical studies of IGFBP-2 by radioimmunoassay

    Blum, W F; Horn, N; Kratzsch, J;

    1993-01-01

    and experimental situations. There was little circadian fluctuations of serum level which showed a marked age-dependence with high levels at birth and senescence and low levels during puberty. Decreased IGFBP-2 levels were present in untreated insulin-dependent diabetes mellitus (IDDM), in acromegaly and during...

  7. SUBJECT INDEX

    1992-01-01

    1, 25-dihydroxyvitamin D3, growth hormone,acromegaly, 9202551-methyl-4phenyl-1, 2, 3, 6-tetra-hydropy-ridine, dyskinesia, 920599201TL planar imaging, myocardial disease,9202165-fluorouracil, pharmacokinetics, 9200165-hydroxyindoleacetic acid, congestive heartfailure, 5-hydroxytryptamine, norepinephrine,9204785-hydroxytryptamine, epidemic hemorrhagicfever, 9200355-hydroxytryptamine, congestive heart failure,

  8. Bromocriptine

    ... hyperprolactinemia caused by certain types of tumors that produce prolactin, and may shrink these tumors. Bromocriptine (Parlodel) is also used alone or with other treatments to treat acromegaly (condition in which there is too much growth hormone in the body) and Parkinson's disease (PD; a ...

  9. Self-injection is a success

    Jensen, Jonna Gintberg; Høi, Henriette Brahe

    Background: 29 of the 120 patients with Acromegaly, which annually comes to control at Aarhus University Hospital, are in lifelong Ipstyl treatment. Ipstyl, which is a viscous composition, is injected with a rather large needle. This requires both mental energy, skill and muscle power. Therefore ...

  10. Interactions between IGFBP-3 and Nuclear Receptors in Prostate Cancer Apoptosis

    2008-01-01

    like growth factor-binding protein-3 levels in the diagnosis of acromegaly. J Clin Endocrinol Metab 80:927–932 19. Alford FP, Hew FL, Christopher MC...14394. Chan JM, Stampfer MJ, Giovannucci E, Gann PH, Ma J, Wilkinson P et al. (1998). Plasma insulin-like growth factor-I and prostate cancer risk: a

  11. Endoscopic transsphenoidal excision of a GH-PRL-secreting pituitary macroadenoma in a patient with McCune-Albright syndrome.

    Natarajan, Manikandan S; Prabhu, Krishna; Chacko, Geeta; Rajaratnam, Simon; Chacko, Ari G

    2012-02-01

    We describe an endoscopic transsphenoidal excision of a GH-PRL-secreting pituitary adenoma and remodeling of frontotemporal fibrous dysplasia in a patient with McCune-Albright syndrome. Sphenoid dysplasia rendered transsphenoidal surgery challenging, but a study of the radiological anatomy and good surgical planning made this feasible. Medical therapy and radiation was required for persistent acromegaly after surgery.

  12. Algorithmic complexity of growth hormone release in humans.

    Prank, K; Wagner, M; Brabant, G

    1997-01-01

    Most hormones are secreted in an pulsatile rather than in a constant manner. This temporal pattern of pulsatile hormone release plays an important role in the regulation of cellular function and structure. In healthy humans growth hormone (GH) secretion is characterized by distinct pulses whereas patients bearing a GH producing tumor accompanied with excessive secretion (acromegaly) exhibit a highly irregular pattern of GH release. It has been hypothesized that this highly disorderly pattern of GH release in acromegaly arises from random events in the GH-producing tumor under decreased normal control of GH secretion. Using a context-free grammar complexity measure (algorithmic complexity) in conjunction with random surrogate data sets we demonstrate that the temporal pattern of GH release in acromegaly is not significantly different from a variety of stochastic processes. In contrast, normal subjects clearly exhibit deterministic structure in their temporal patterns of GH secretion. Our results support the hypothesis that GH release in acromegaly is due to random events in the GH-producing tumorous cells which might become independent from hypothalamic regulation.

  13. Bilateral carpal tunnel syndrome in Graves' disease.

    Manganelli, P; Pavesi, G; Salaffi, F

    1987-01-01

    Carpal tunnel syndrome (CTS) may be associated with endocrinopathies, such as hypothyroidism and acromegaly. A direct relationship between CTS and hyperthyroidism has recently been suggested. We now report a case in which bilateral CTS developed after treatment of Graves' disease, thus, questioning the possibility of a relationship between these two disease processes.

  14. Lanreotide Injection

    Lanreotide injection is used to treat people with acromegaly (condition in which the body produces too much growth hormone, causing enlargement of the hands, feet, and facial features; joint pain; and other symptoms) who have not successfully, or cannot be treated ...

  15. Management of endocrine disease: GH excess: diagnosis and medical therapy

    Andersen, Marianne

    2014-01-01

    for acromegaly. Papers published on pasireotide have reported an increased risk of diabetes mellitus due to a reduction in insulin levels.Pegvisomant, the GH receptor antagonist, is indicated - alone or in combination with a somatostatin analogue - in most patients who fail to enter remission on a somatostatin...

  16. Intensive Multidiscipline Co-operation, Enhanced Diagnosis and Management of Acromegaly——Interpretation of "Chinese Guideline of Treatment and Management of Acromegaly, 2013"%加强多学科协作,努力提高肢端肥大症诊治水平——《中国肢端肥大症诊治指南(2013版)》解读

    雷霆; 徐钰; 陈娟

    2013-01-01

    由中华医学会内分泌学分会和神经外科学分会、中国垂体瘤协作组共同组织全国众多专家修订的《中国肢端肥大症诊治指南(2013版)》公开发表.本次对指南有较多关键修改之处,主要表现在几个方面:促进多学科交叉,早期诊断和治疗;影响手术治疗效果的主要因素;药物及其他治疗;深入探讨垂体生长激素腺瘤发病机制的重要性.

  17. 中国肢端肥大症诊治规范(草案)——中华医学会内分泌学分会中华医学会神经外科学分会%Diagnosis and treatment standards of acromegaly in China (rough draft) Endocrinology Branch of Chinese Academy of Medical Sciences,Neurosurgery Branch of Chinese Academy of Medical Sciences

    中华医学会内分泌学分会; 中华医学会神经外科学分会

    2006-01-01

    @@ 1 前言 肢端肥大症(以下简称"肢大")是一种起病隐匿的慢性进展性内分泌疾病,患者就诊时病程可能已达数年甚至10年以上.该病的主要特征是体内产生过量生长激素(growth hormone,GH).

  18. Cardiovascular risk factors in patients with uncontrolled and long-term acromegaly: Comparison with method data from the general population and the effect of diseases control%长期未获控制的肢端肥大症患者的心血管危险因素:与普通人群中条件匹配者的比较以及疾病控制的效果

    赵红燕

    2010-01-01

    @@ 肢端肥大症的特点为生长激素(GH)和胰岛素样生长因子Ⅰ(IGF-Ⅰ)长期分泌过多,导致多种疾病,主要是心脑血管病的发病率和死亡率增加.患者常因肥胖、高血压、血糖或血脂异常等合并症而处于心血管疾病高危状态.尽管有上述不利因素,但仍不清楚这些患者是否存在冠心病(CHD)危险,以及风险的大小.迄今为止,有关肢端肥大症患者心血管危险和CHD的资料主要来自性质不同的患者群体以及过去病例系列的病理检查结果.尸检资料显示在长期肢端肥大症患者中可见冠状动脉粥样硬化.Courville和Mason在接受尸检的肢端肥大症患者中发现,24%有主动脉粥样硬化,11%有冠状动脉疾病,15%有陈旧性心肌梗死.还有一些研究通过超声检查发现50%的肢端肥大症患者过早出现颈动脉粥样硬化伴内膜中层增厚,而在疾病得到控制后可恢复正常.

  19. Giant growth-hormone secreting pituitary tumour with etracranial extension

    Ip Taipang; Chan Fuluk; Kung Annie Waichee; Lam Karen Siuling [Univ. of Hong Kong, Queen Mary Hospital (Hong Kong). Depts. of Medicine and Diagnostic Radiology

    1996-02-01

    A 19 year old female patient with typical features of acromegaly was found to have an extensive pituitary tumour with suprasellar, lateral and inferior extensions. Magnetic resonance imaging (MRI) also showed a portion of the tumour extending from the right cavernous sinus through the foramen ovale to become extracranial. Serum growth hormone (GH) was 52.6 mU/L basally and remained elevated after oral glucose, confirming the diagnosis of acromegaly. Treatment with the long-acting somatostatin analogue, octreotide, for 6 months led to a 30% reduction in tumour volume of the intracranial portion but no effect on the extracranial and sphenoidal extensions. She was subsequently treated with trans-sphenoidal surgery followed by external irradiation. The possibility of perineural spread of the tumour was considered. 9 refs., 1 tab., 1 fig.

  20. Into the wardrobe of Narnia: beyond HIV infection a world of cardiovascular risk.

    Colotto, Marco; Renzi, Alessandra; Durante, Cosimo

    2012-07-17

    A 38-years-old HIV-hepatitis C virus (HCV) coinfected woman presented to us for dyslipidemia. Clinical, familial and laboratory data were consistent with the diagnosis of familial heterozygous hypercholesterolaemia. History, clinical examination and laboratory findings also supported suspected acromegaly. A pituitary MRI showed a sellar macroadenoma that was removed by transphenoidal surgery without complications. MRI carried out 6 months after surgery showed almost complete removal of the adenoma. This case report describes an uncommon association of diseases, very different for aetiology--genetic for FH, infectious for HIV and HCV, caused by excessive hormone secretion for acromegaly--clinical features and therapy, but all sharing a significant impact on cardiovascular risk as a common feature.