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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. 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.

  3. 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.

  4. 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.

  5. Voice characteristics of acromegaly.

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

    2013-03-01

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

  6. 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.

  7. Sleep apnoea in acromegaly.

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

    1980-01-01

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

  8. 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.

  9. 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.

  10. Echocardiographic Findings in 11 Cats with Acromegaly

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

    2014-01-01

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

  11. Pasireotide in Acromegaly: A Review.

    McKeage, Kate

    2015-06-01

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

  12. Homocysteine levels in acromegaly patients.

    Hekimsoy, Zeliha; Ozmen, Bilgin; Ulusoy, Sadik

    2005-12-01

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

  13. Development of acromegaly in patients with prolactinomas

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

    2003-01-01

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

  14. Acromegaly

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

  15. Acromegaly

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

  16. Acromegaly

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

  17. Urinary growth hormone excretion in acromegaly

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

    1993-01-01

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

  18. Sequential growth hormone deficiency and acromegaly.

    Heffernan, A.

    1988-01-01

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

  19. 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

  20. 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=...

  1. Insulin action in human adipose tissue in acromegaly.

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

    1986-01-01

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

  2. Acromegaly and diabetes mellitus associated with hyperthyroidism.

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

    2014-01-01

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

  3. MRI of pituitary adenomas in acromegaly

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

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

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

  5. The Role of Radiotherapy in Acromegaly.

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

    2016-01-01

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

  6. 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.

  7. 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

  8. 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

  9. Acute exacerbation of upper airway obstruction in acromegaly

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

    1982-01-01

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

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

    Nessrine Akasbi

    2011-10-01

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

  11. 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...

  12. 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

  13. 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.

  14. 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.

  15. 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

  16. 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.

  17. Pregnancy in acromegaly patients treated with pegvisomant.

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

    2015-08-01

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

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

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

    2012-09-01

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

  19. 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

  20. 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.

  1. Body composition in acromegaly - before and after therapy

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

  2. Acromegaly Quality of Life Questionnaire (AcroQoL

    Lara Nuria

    2004-02-01

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

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

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

    2016-05-01

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

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

    Boguszewski, Cesar Luiz; Ayuk, John

    2016-10-01

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

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

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

    2016-04-01

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

  6. The Modern Criteria for Medical Management of Acromegaly.

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

    2016-01-01

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

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

    Bonert, Vivien

    2009-12-01

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

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

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

    2016-01-01

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

  9. 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

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

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

    2012-01-01

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

  11. 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

  12. 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.

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

    Christofides, Elena A

    2016-01-01

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

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

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

    2008-01-01

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

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

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

    2005-01-01

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

  16. 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.

  17. 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.)

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

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

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

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

    2016-01-01

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

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

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

    2016-08-01

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

  1. 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.

  2. 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

  3. 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.

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

    Jain Kapil; Jain V; Aggarwal Kamal; Bansal Anu

    2008-01-01

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

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

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

    2016-01-01

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

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

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

    2003-01-01

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

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

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

    2015-12-01

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

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

    Julia Szendroedi

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

  9. 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.

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

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

    2015-01-01

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

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

    Rosario, Pedro Weslley; Calsolari, Maria Regina

    2012-06-01

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

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

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

    2012-01-01

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

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

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

    2009-01-01

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

  14. 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.

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

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

    2015-06-01

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

  16. 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.

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

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

    2016-04-25

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

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

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

    1983-01-01

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

  19. 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.)

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

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

    2016-07-18

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

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

    Bonert Vivien S

    2010-05-01

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

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

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

    2002-01-01

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

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

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

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

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

    2010-01-01

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

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

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

    2014-01-01

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

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

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

    2010-01-01

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

  7. 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.

  8. 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.

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

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

    2003-07-01

    Stereotactic radiosurgery by gamma-knife (GK) is an attractive therapeutic option after failure of microsurgical removal in patients with pituitary adenoma. In these tumors or remnants of them, it aims to obtain the arrest of cell proliferation and hormone hypersecretion using a single precise high dose of ionizing radiation, sparing surrounding structures. The long-term efficacy and toxicity of GK in acromegaly are only partially known. Thirty acromegalic patients (14 women and 16 men) entered a prospective study of GK treatment. Most were surgical failures, whereas in 3 GK was the primary treatment. Imaging of the adenoma and target coordinates identification were obtained by high resolution magnetic resonance imaging. All patients were treated with multiple isocenters (mean, 8; range, 3-11). The 50% isodose was used in 27 patients (90%). The mean margin dose was 20 Gy (range, 15-35), and the dose to the visual pathways was always less than 8 Gy. After a median follow-up of 46 months (range, 9-96), IGF-I fell from 805 micro g/liter (median; interquartile range, 640-994) to 460 micro g/liter (interquartile range, 217-654; P = 0.0002), and normal age-matched IGF-I levels were reached in 7 patients (23%). Mean GH levels decreased from 10 micro g/liter (interquartile range, 6.4-15) to 2.9 micro g/liter (interquartile range, 2-5.3; P < 0.0001), reaching levels below 2.5 micro g/liter in 11 (37%). The rate of persistently pathological hormonal levels was still 70% at 5 yr by Kaplan-Meier analysis. The median volume was 1.43 ml (range, 0.20-3.7). Tumor shrinkage (at least 25% of basal volume) occurred after 24 months (range, 12-36) in 11 of 19 patients (58% of assessable patients). The rate of shrinkage was 79% at 4 yr. In no case was further growth observed. Only 1 patient complained of side-effects (severe headache and nausea immediately after the procedure, with full recovery in a few days with steroid therapy). Anterior pituitary failures were observed in 2 patients

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

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

    2007-01-01

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

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

    Losa, Marco; Bollerslev, Jens

    2016-06-01

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

  12. 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.

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

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

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

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

    1990-09-01

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

  15. 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

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

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

    2010-01-01

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

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

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

    2012-01-01

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

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

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

    2010-01-01

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

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

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

    2014-01-01

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

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

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

    2016-07-01

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

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

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

    2007-01-01

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

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

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

    2016-06-01

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

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

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

    2012-01-01

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

  5. 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

  6. 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

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

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

    2000-01-01

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

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

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

    2001-01-01

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

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

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

    2012-01-01

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

  10. Acromegaly (Beyond the Basics)

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

  11. 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.

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

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

    2001-01-01

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

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

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

  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. 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

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

    Gomez-Panzani E

    2012-12-01

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

  17. Patients' experience of nursing with Acromegaly

    Jensen, Jonna Gintberg; Høi, Henriette Brahe

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

  18. ontaneous Remission of Acromegaly Due to Apoplexy

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

    2008-01-01

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

  19. New Insights into Medical Treatment of Acromegaly

    Neggers, Bas

    2011-01-01

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

  20. 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....

  1. Beta-adrenoceptor-blocking drugs, growth hormone and acromegaly.

    Feely, J.

    1980-01-01

    Chronic treatment with oxprenolol or propranolol in active hypertensive patients was associated with elevation of serum growth hormone (GH). Propranolol, 80 mg orally, caused a marked rise in GH in 3 of 4 acromegalic patients.

  2. 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...

  3. Pituitary macroadenoma presenting with pituitary apoplexy, acromegaly and secondary diabetes mellitus - a case report.

    Nganga, Hudson Kamau; Lubanga, Reuben Paul

    2013-01-01

    Pituitary adenomas are associated with significant morbidity. The usual symptoms on presentation are of endocrine dysfunction and mass effects. A 31-year-old African female presented with headache, irregular menses, blurring of vision in the right eye and complete loss of vision in the left eye for 1 year. She had coarse facial features, enlarged hands and feet. Her right eye had temporal hemianopia with decreased visual acuity and her left eye had no perception of light. Investigations revealed an elevated fasting blood sugar and an elevated prolactin and growth hormone level. A CT scan and MRI done showed a hemorrhagic pituitary macroadenoma. She was put on bromocriptine, ocreotide, analgesics and insulin. Thereafter, she underwent transphenoidal surgery, where near total resection of the tumor was achieved. Patient is doing well post-operatively. This case highlights the importance of the use of a high clinical index of suspicion and radiological findings in diagnosis. PMID:24062868

  4. Pituitary macroadenoma presenting with pituitary apoplexy, acromegaly and secondary diabetes mellitus - a case report

    Nganga, Hudson Kamau; Lubanga, Reuben Paul

    2013-01-01

    Pituitary adenomas are associated with significant morbidity. The usual symptoms on presentation are of endocrine dysfunction and mass effects. A 31-year-old African female presented with headache, irregular menses, blurring of vision in the right eye and complete loss of vision in the left eye for 1 year. She had coarse facial features, enlarged hands and feet. Her right eye had temporal hemianopia with decreased visual acuity and her left eye had no perception of light. Investigations revea...

  5. Craniofacial abnormalities and their relevance for sleep apnoea syndrome aetiopathogenesis in acromegaly

    Dostálová, S.; Šonka, K.; Šmahel, Zbyněk; Weiss, V.; Marek, J.; Hořínek, D.

    2001-01-01

    Roč. 144, - (2001), s. 491-497. ISSN 0804-4643 R&D Projects: GA MZd IZ3575 Institutional research plan: CEZ:AV0Z5039906 Keywords : apnoea syndrome Subject RIV: AC - Archeology, Anthropology, Ethnology Impact factor: 2.133, year: 2001

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

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

    2015-10-01

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

  7. 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.

  8. 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.

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

    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)

  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. Growth hormone responses to hp GRF 1-44 amide, bromocriptine and stress in acromegaly are correlated.

    Belchetz, P E

    1987-01-01

    The results of testing growth hormone (GH) reserve using human pancreatic growth hormone-releasing factor 1-44 amide (hp GRF 1-44 amide) have been compared with the GH responses in a variety of other dynamic tests in seven acromegalic patients. The GH release following hp GRF 1-44 amide correlated with the GH suppression following bromocriptine, but showed an inverse correlation with the GH release following stress tests (insulin-induced hypoglycaemia/glucagon). There was no correlation betwe...

  12. Quantitative analyses of T2-weighted MRI as a potential marker for response to somatostatin analogs in newly diagnosed acromegaly.

    Heck, Ansgar; Emblem, Kyrre E; Casar-Borota, Olivera; Bollerslev, Jens; Ringstad, Geir

    2016-05-01

    In growth hormone (GH)-producing adenomas, T2-weighted MRI signal intensity is a marker for granulation pattern and response to somatostatin analogs (SSA). Prediction of treatment response is necessary for individualized treatment, and T2 intensity assessment might improve preoperative classification of somatotropinomas. The objectives of this study are (I) to explore the feasibility of quantitative T2-weighted MRI histogram analyses in newly diagnosed somatotroph adenomas and their relation to clinical and histological parameters and (II) to compare the quantitative method to conventional, visual assessment of T2 intensity. The study was a retrospective cohort study of 58 newly diagnosed patients. In 34 of these, response to primary SSA treatment after median 6 months was evaluated. Parameters from the T2 histogram analyses (T2 intensity ratio and T2 homogeneity ratio) were correlated to visually assessed T2 intensity (hypo-, iso-, hyperintense), baseline characteristics, response to SSA treatment, and histological granulation pattern (anti-Cam5.2). T2 intensity ratio was lowest in the hypointense tumors and highest in the hyperintense tumors (0.66 ± 0.10 vs. 1.07 ± 0.11; p treatment (n = 34). The T2 homogeneity ratio correlated with adenoma size reduction (r = -0.45; p = 0.008). Sparsely granulated adenomas had a higher T2 intensity than densely or intermediately granulated adenomas. T2 histogram analyses are an applicable tool to assess T2 intensity in somatotroph adenomas. Quantitatively assessed T2 intensity ratio in GH-producing adenomas correlates with conventional assessment of T2 intensity, baseline characteristics, response to SSA treatment, and histological granulation pattern. PMID:26475495

  13. 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

  14. 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...

  15. 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...

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

    2016-02-28

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

  17. 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....

  18. 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.

  19. 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...

  20. Stages of Pituitary Tumors

    ... arms. Bones that break easily. Anxiety , irritability, and depression . Too much growth hormone may cause: Headache. Some loss of vision. In adults, acromegaly (growth of the bones in the face, ...

  1. General Information about Pituitary Tumors

    ... arms. Bones that break easily. Anxiety , irritability, and depression . Too much growth hormone may cause: Headache. Some loss of vision. In adults, acromegaly (growth of the bones in the face, ...

  2. Treatment Option Overview (Pituitary Tumors)

    ... arms. Bones that break easily. Anxiety , irritability, and depression . Too much growth hormone may cause: Headache. Some loss of vision. In adults, acromegaly (growth of the bones in the face, ...

  3. McCune-Albright syndrome

    ... bones in the face Gigantism Irregular, large patchy cafe-au-lait spots , especially on the back ... Acromegaly Adrenal abnormalities Gigantism Hyperparathyroidism Hyperthyroidism Hypophosphatemia Large cafe-au-lait spots on the skin Liver disease, ...

  4. GH and the cardiovascular system: an update on a topic at heart

    Isgaard, Jörgen; Arcopinto, Michele; Karason, Kristjan; Cittadini, Antonio

    2014-01-01

    In this review, the importance of growth hormone (GH) for the maintenance of normal cardiac function in adult life is discussed. Physiological effects of GH and underlying mechanisms for interactions between GH and insulin-like growth factor I (IGF-I) and the cardiovascular system are covered as well as the cardiac dysfunction caused both by GH excess (acromegaly) and by GH deficiency in adult hypopituitary patients. In both acromegaly and adult GH deficiency, there is also increased cardiova...

  5. 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....

  6. 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.

  7. 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.

  8. 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. PMID:1946104

  9. Growth hormone and prolactin radioimmunoassay in early diagnosis of pituitary tumors

    Results of prolactin and HGH determination in basal conditions and following stimulation tests in the group of 68 patients with pituitary or suprasellar tumors are presented. In acromegaly elevated level of HGH in fasting state, lack of supression after glucose loading and parodoxical drop of HGH after L-dopa administration were observed. In pituitary tumors without acromegaly determinations of HGH during insulin induced hypoglycemia revealed lack of HGH response to such stimulation in 25 cases which indicated hypopituitarism. In 10 cases elevated prolactin levels (48 - 1000 ng/ml) were observed, this indicates that some of so-called inactive tumors are in fact hormonally active. (author)

  10. Pituitary: Secretory Tumors

    ... is caused by the excess secretion of growth hormone (GH). It can cause noticeable changes in your appearance, ... medication approved for acromegaly. Instead of suppressing excess GH production by the pituitary tumor, it works to stop the hormone from acting on the body, but does not ...

  11. Basal, But Not Pulsatile, Growth Hormone Secretion Determines the Ambient Circulating Levels of Insulin-Like Growth Factor-I

    Faje, Alexander T.; Barkan, Ariel L.

    2010-01-01

    Context: Previous studies have shown that mean 24-h GH concentrations determine plasma IGF-I levels in patients with acromegaly. However, we have recently shown that continuous GH infusion, mimicking the interpulse GH levels, was significantly more effective than the pulsatile GH administration at increasing IGF-I concentrations.

  12. Primary Cardiac Lymphoma: Helical CT Findings and Radiopathologic Correlation

    Primary tumors of the heart are extremely rare.Clinical manifestations are nondiagnostic and the patients are often misdiagnosed. Magnetic resonance imaging and echocardiography are standard in this diagnostic workup. We report a case of a man with acromegaly, dysphagia, chest pain and weight loss. An invasive cardiac mass was diagnosed by helical-CT. Autopsy demonstrated a B-cell aggressive lymphoma

  13. Deficiencies in fat-soluble vitamins in long-term users of somatostatin analogue

    Fiebrich, H. -B.; van den Berg, G.; Kema, I. P.; Links, T. P.; Kleibeuker, J. H.; van Beek, A. P.; Walenkamp, A. M. E.; Sluiter, W. J.; de Vries, E. G. E.

    2010-01-01

    P>Background Somatostatin analogues are administered to control hormone hypersecretion in acromegaly and carcinoid patients. Somatostatin analogues can increase fat in the stools, which can lead to loss of fat-soluble vitamins. The effect of long-term somatostatin analogue use on vitamin levels rema

  14. Pituitary irradiation program

    The alpha particle pituitary irradiation program continues to be a major research project at Donner Pavilion. A study to determine the incidence of hyperprolactinemia in a large series of acromegalic subjects was undertaken. The relationships between plasma levels of growth hormone and prolactin, sellar volume, duration of acromegaly, and age at time of evaluation were investigated

  15. Bromocriptine, a Dopamine (d2) Receptor Agonist, Used Alone and in Combination with Glipizide in Sub–Therapeutic Doses to Ameliorate Hyperglycaemia

    Kumar V.S., Harish; M.B., Vinutha; A.N., Pradeep; Aithal, Sathisha; Baleed, Sindhura Reddy; Patil, Umakant N

    2013-01-01

    Introduction: Bromocriptine, an ergot derivative, is an agonist at the dopamine 2 receptor and a sympatholytic. It is a well established drug in Parkinsonism, hyperprolactinaemia and acromegaly and it has various other clinical indications like induction of ovulation in female infertility. Bromocriptine has been evaluated in alloxan induced diabetic rats for its anti-hyperglycaemic effect with and without simultaneous use of glipizide.

  16. 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 ...

  17. 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,

  18. Mental retardation, acromegalic face, and megalotestes in two half-brothers: a specific form of X-linked mental retardation without fra(X) (q)?

    Tariverdian, G; Froster-Iskenius, U; Deuschl, G; Wolff, G

    1991-01-01

    We describe a family with two half-brothers affected with severe mental retardation. The phenotype in the affected individuals is characterized by apparent acromegaly, profound mental retardation, and hyperactivity. The mother has analogous but less severe facial anomalies and mild mental impairment. Screening for fra(X) (q) was negative in peripheral lymphocytes using methotrexate for fra(X) enhancement. The clinical findings in our patients are similar to those described by Fryns et al. [1986] in two patients with acquired lesions of the central nervous system. CT investigations in one of our patients showed areas of hyperdensity in the pontine region and a small subarachnoid cyst. The pedigree suggests X-linked inheritance. The association of apparent acromegaly, CNS anomalies, megalotestes, and mental retardation in this family supports the hypothesis that a distinct syndrome may exist with phenotype anomalies more severe than those characteristic for the Martin-Bell syndrome but without fragile X. PMID:2018059

  19. Las manifestaciones orales de acromegalia

    Acromegaly has been a disease caused in most cases by a benign pituitary tumor secreting growth hormone. A series of physical and metabolic changes are produced, shorting the lifespan of the sufferer and also the risk of other diseases is increased. The prevalence of this disease has been of 40-90 cases per million of person. Usually, a very slow course has contributed to a high rate of misdiagnosis. Within its manifestations have arisen some of odontological origin and their prognathism, separation of teeth, macroglossia, and poor occlusion. The odontologist must consider the possibility of acromegaly when a patient has presented with some manifestations. The diagnosis is confirmed by elevated levels of growth hormones and growth factor similar to type 1 insulin. (author)

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

    From 1976 till 1981 56 patients with pituitary tumors were observed. From a total of 51 adenomas 17 prolactinomas (33.3%), 15 acromegalies (29.4%), 4 Cushing-syndromes (7.8%), 1 TSH-producing adenoma (1.9%) and 14 hormone-inactive adenomas (27.4%) were found. In addition, there were 4 craniopharyngeomas and 1 intrasellar meningeoma. For the individual types of adenomas prae- and postoperative hormonal data after transcranial resp. transsphenoidal adenomectomy are listed. Prolactinomas responded well to bromocriptine therapy in some cases, whereas acromegalies showed no remission after bromocriptine. After radiotherapy of 4 recidive-adenomas remission in one case was seen. Problems and objects of long-term-care of patients with pituitary tumors are discussed. (author)

  1. Carpal Fusion

    Jalal Jalalshokouhi; Mohammad Hossein Herischi; Shahyar Pashaei; Ali Akbar Ameri

    2012-01-01

    Carpal fusion may be seen in hereditary and nonhereditary conditions such as acrocallosal syndrome,acromegaly, Apert syndrome, arthrogryposis, Carpenter syndrome, chromosomal abnormalities, ectrodactyly-ectodermal dysplasia-cleft (EEC) syndrome, the F form of acropectorovertebral dysgenesis or the F syndrome, fetal alcohol syndrome, Holt-Oram syndrome, Leopard syndrome, multiple synostosis syndrome, oligosyndactyly syndrome, Pfeiffer-like syndrome, scleroderma, split hand and foot malformatio...

  2. Silent somatotroph adenomas of the human pituitary. A morphologic study of three cases including immunocytochemistry, electron microscopy, in vitro examination, and in situ hybridization.

    Kovacs, K.; Lloyd, R.; Horvath, E.; Asa, S L; Stefaneanu, L.; Killinger, D W; Smyth, H. S.

    1989-01-01

    Pituitary adenomas, removed surgically from three women with normal or slightly elevated serum growth hormone levels and no evidence of acromegaly, were studied. The tumor cells were shown by electron microscopy to correspond to sparsely granulated somatotrophs but immunocytochemistry showed that they contained no, moderate, or little growth hormone. Two tumors examined in vitro secreted small amounts of growth hormone in the tissue culture medium initially with a spontaneous rise after sever...

  3. Growth hormone and its disorders

    Ayuk, J.; Sheppard, M C

    2006-01-01

    Growth hormone (GH) is synthesised and secreted by the somatotroph cells of the anterior lobe of the pituitary gland. Its actions involve multiple organs and systems, affecting postnatal longitudinal growth as well as protein, lipid, and carbohydrate metabolism. GH hypersecretion results in gigantism or acromegaly, a condition associated with significant morbidity and mortality, while GH deficiency results in growth retardation in children and the GH deficiency syndrome in adults. This articl...

  4. STAT3 upregulation in pituitary somatotroph adenomas induces growth hormone hypersecretion

    Zhou, Cuiqi; Jiao, Yonghui; Wang, Renzhi; Ren, Song-Guang; Wawrowsky, Kolja; Melmed, Shlomo

    2015-01-01

    Pituitary somatotroph adenomas result in dysregulated growth hormone (GH) hypersecretion and acromegaly; however, regulatory mechanisms that promote GH hypersecretion remain elusive. Here, we provide evidence that STAT3 directly induces somatotroph tumor cell GH. Evaluation of pituitary tumors revealed that STAT3 expression was enhanced in human GH-secreting adenomas compared with that in nonsecreting pituitary tumors. Moreover, STAT3 and GH expression were concordant in a somatotroph adenoma...

  5. Scalloping at the lumbosacral canal

    Reinhardt, R.

    1987-07-01

    Scalloping is an indentation of the dorsal side of the vertebral body (anterior wall of the lumbosacral or sacral canal) which typically involves several adjacent lumbal vertebral body segments and the anterior wall of the canalis sacralis. Occurrence without underlying disease is rare; it occurs most frequently with chondrodystrophy, neurofibromatosis, Morquio's syndrome, Hurler's syndrome, acromegaly, Ehlers-Danlos syndrome, Marfan's syndrome, cysts, tumors and in peridural lipomas.

  6. Scalloping at the lumbosacral canal

    Scalloping is an indentation of the dorsal side of the vertebral body (anterior wall of the lumbosacral or sacral canal) which typically involves several adjacent lumbal vertebral body segments and the anterior wall of the canalis sacralis. Occurrence without underlying disease is rare; it occurs most frequently with chondrodystrophy, neurofibromatosis, Morquio's syndrome, Hurler's syndrome, acromegaly, Ehlers-Danlos syndrome, Marfan's syndrome, cysts, tumors and in peridural lipomas. (orig.)

  7. Immunohistochemical detection of dopamine D2 receptors in human pituitary adenomas.

    Marek Pawlikowski

    2010-01-01

    Thirty one pituitary adenomas and 3 samples of peritumoral anterior pituitary tissue were immunostained with an antibody raised against dopamine D2 receptor protein. The positive reactions were found in cell cytoplasm, a subpopulation of cell nuclei and the intratumoral blood vessels walls. As expected, the positive immunostaining was shown in cytoplasm and/or cell nuclei of all examined prolactinomas (7/7). In acromegaly the positive D2 staining occurred in 5/7 samples, in gonadotropinomas i...

  8. The seminal role played by Pierre Marie in Neurology and Internal Medicine

    Gustavo M Almeida

    2015-10-01

    Full Text Available The authors review the most important contributions of Pierre Marie to the elucidation and description of several neurological diseases, such as Charcot-Marie-Tooth’s disease and hereditary cerebellar ataxia, as well as his contributions to Internal Medicine, including his pioneering studies on acromegaly, ankylosing spondylitis, and hypertrophic pulmonary osteoarthropathy. His works led to incontestable advances in the medical sciences that transcended his time.

  9. Berardinelli–Seip syndrome: highlight of treatment challenge

    Ferraria, Nélia; Pedrosa, Cristina; Amaral, Daniela; Lopes, Lurdes

    2013-01-01

    Berardinelli–Seip congenital lipodystrophy (BSCL) syndrome is a rare autosomal-recessive disease characterised by lipoatrophy and associated with deregulations of glycidic and lipid metabolism. We report three BSCL cases with its typical clinical picture and complications. Clinically, they all show marked atrophy of adipose tissue, acromegaly, acanthosis nigricans and tall stature. Two cases present attention deficit hyperactivity and developmental learning disorders; another patient has hype...

  10. Experience from the Argentine Pegvisomant Observational Study: preliminary data.

    García Basavilbaso, N; Guitelman, M; Nagelberg, A; Stalldecker, G; Carabelli, A; Bruno, O; Danilowitz, K; Manavela, M; Mallea Gil, S; Ballarino, C; Guelman, R; Katz, D; Fidalgo, S; Leal, R; Fideleff, H; Servidio, M; Bruera, D; Librandi, F; Chervin, A; Vitale, M; Basso, A

    2010-01-01

    The GH receptor antagonist pegvisomant is an efficient agent to achieve biochemical control of acromegaly in those cases refractory to surgery and medical therapy with somatostatin analogs. We conducted an observational multicenter study consisting of data collection in accordance with the standard management of patients with acromegaly in everyday practice. We reviewed the medical records of 28 patients, 23 females, who were treated with pegvisomant due to the lack of biochemical response or intolerance to the somatostatin analogs. The objective was to monitor long-term safety and efficacy of the antagonist. 82% of the patients had previous pituitary surgery, 53.6% radiotherapy and 96.4% received medical therapy for acromegaly. Only 19.2% of the patients had pituitary residual tumor size larger than 1 cm, the remainder harbored a microadenoma or no visible tumor in the pituitary images. In terms of biochemical efficacy, IGF-I levels decreased to normal ranges in 45% and 58.8% of patients after 3 and 6 months of treatment, respectively, the daily mean dose of pegvisomant being 9.6+/-1.1 mg. Adverse events, potentially related to pegvisomant were reported in 6 patients (21.4%), local injection site reaction and elevated liver enzymes being the most frequent. Tumor size did not show enlargement in the evaluated population (15 patients) during the period of the study. This paper presents preliminary data from a small observational study in Argentina which represents the first database in our country. PMID:20616494

  11. Lanreotide depot deep subcutaneous injection: a new method of delivery and its associated benefits

    Carmichael JD

    2012-01-01

    Full Text Available John D CarmichaelDepartment of Medicine, Cedars-Sinai Medical Center, Los Angeles, CA, USAAbstract: Acromegaly is a rare disease characterized by excessive growth hormone secretion, usually from a pituitary tumor. Treatment options include surgery, medical therapy, and in some cases, radiation therapy. Current medical therapy consists of treatment with somatostatin analog medications or a growth hormone receptor antagonist. There are two somatostatin analogs currently in use, octreotide and lanreotide. Both are supplied in long-acting formulations and are of comparable biochemical efficacy. Lanreotide is supplied in a prefilled syringe and is injected into deep subcutaneous tissue. Studies have been conducted to assess the efficacy of self- or partner administration, and have demonstrated that injection of lanreotide can be accomplished reliably and safely outside a physician's office. For patients who have achieved biochemical control with lanreotide, the FDA has recently approved an extended dosing interval. Selected patients may be able to receive the medication less frequently with injections of 120 mg administered every 6 or 8 weeks. This review focuses on the use of lanreotide in the treatment of acromegaly, the safety and efficacy of the drug, and the benefits afforded to patients because of unique aspects of the delivery of lanreotide.Keywords: acromegaly, treatment, lanreotide, somatostatin analog, pituitary tumor

  12. Prostatic disorders in acromegalic patients experience of a Brazilian center

    Livia L. Correa

    2013-06-01

    Full Text Available Introduction Published data suggest that patients with acromegaly have an increased prevalence of prostate disorders. Objective To evaluate prostatic disorders in acromegalic patients comparing these results after one year of treatment of acromegaly and with a group of healthy men. Materials and Methods This study was composed of two parts: sectional study comparing patients with healthy controls (baseline and prospective, longitudinal study (at baseline and after one year of treatment. Forty acromegalic patients were enrolled and evaluated at baseline and after one year with the application of international prostatic symptoms score (IPSS, digital rectal examination, measurements of growth hormone (GH, insulin-like growth factor-I (IGF-I, insulin-like growth factor-binding protein-3 (IGFBP-3, sex hormone-binding globulin (SHBG, prolactin, luteinizing hormone (LH, follicle-stimulating hormone (FSH, total testosterone, total and free prostate-specific antigen (PSA levels and prostate ultrasonography (US. Thirty healthy men were selected as control group. Results We stratified patients and controls according to age, considering 40 years-old as cut off. Healthy controls under 40 had IPSS values lower than acromegalic patients. When considering only older patients and controls prostate hyperplasia and structural abnormalities were more frequent in acromegalics. After one year of treatment there was significant decrease in GH, IGF-I and prostate volume in acromegalics over 40 years-old. Conclusions Acromegalics under 40 have more urinary symptoms according to IPSS and above 40 years-old higher frequency of structural changes and increased prostate volume than healthy men. Significant reduction of GH and IGF-I levels during treatment of acromegaly leads to decrease in the prostate volume.

  13. Alcohol septal ablation in obstructive acromegalic hypertrophic cardiomyopathy - a first case report.

    Viveiros Monteiro, André; Fiarresga, António; Cacela, Duarte; de Sousa, Lídia; Ramos, Ruben; Galrinho, Ana; Branco, Luísa; Cruz Ferreira, Rui

    2016-09-01

    Acromegaly is a rare disease, mostly caused by a growth hormone (GH)-secreting benign pituitary tumor, with an increased production of GH and insulin-like growth factor 1 (IGF-1). Cardiovascular complications are common and are associated with cardiomyocyte apoptosis and concentric cardiac hypertrophy. Suppression of GH and IGF-1 appears to improve cardiac function only in the short term, with little or no decrease in left ventricular mass or improvement in cardiac function after prolonged treatment. Alcohol septal ablation (ASA) has emerged as a minimally invasive alternative to septal myectomy, with significant improvement in symptoms, gradients and left ventricular remodeling. In this report, we describe the case of a 73-year-old woman with acromegaly due to a pituitary adenoma diagnosed and treated surgically at the age of 38 but with recurrence and reoperation at the age of 50. She was referred to our cardiology department due to a three-month history of progressively worsening exercise-induced dyspnea and orthopnea under optimal medical therapy. Echocardiography and magnetic resonance imaging revealed severe basal hypertrophy of the interventricular septum (19 mm), dynamic left ventricular outflow tract obstruction with a gradient of 70 mmHg at rest and 120 mmHg with Valsalva maneuver, and systolic anterior movement (SAM). Genetic testing excluded the most frequent forms of familial hypertrophic cardiomyopathy. ASA was performed with injection of 2 cc of alcohol in the first septal branch of the left coronary artery, without complications. At one-year reassessment, significant clinical and echocardiographic improvement was noted, with disappearance of SAM. To our knowledge, there have been no previously reported cases of ASA in hypertrophic cardiomyopathy due to acromegaly. We report a case of successful ASA in acromegalic cardiomyopathy. PMID:27503591

  14. Comparing acromegalic patients to healthy controls with respect to intraocular pressure, central corneal thickness, and optic disc topography findings

    Emine Sen

    2014-01-01

    Full Text Available Aims: The aim was to compare the intraocular pressure (IOP, central corneal thickness (CCT, and optic disc topography findings of biochemically controlled acromegalic patients and the control group and to evaluate the effect of the duration of acromegaly and serum growth hormone and insulin-like growth factor-1 (IGF-1 levels on these ocular parameters. Materials and Methods: IOP measurement with Goldmann applanation tonometry, CCT measurement with ultrasonic pachymetry, and topographic analysis with Heidelberg retinal tomograph III were performed on 35 biochemically controlled acromegalic patients and 36 age- and gender-matched controls. Results: Mean IOP and CCT were 14.7 ± 2.9 mmHg and 559.5 ± 44.9 μm in the acromegaly patients and 13.0 ± 1.6 mmHg and 547.1 ± 26.7 μm in controls (P = 0.006 and P = 0.15, respectively. A significant moderate correlation was found between the duration of acromegaly and CCT (r = 0.391 and IOP (r = 0.367. Mean retinal nerve fiber layer (RNFL thickness was significantly lower in the acromegalic patients (0.25 ± 0.05 mm as compared to controls (0.31 ± 0.09 mm (P = 0.01. A significant moderate correlation was detected between IGF-1 level and disc area (r = 0.362, cup area (r = 0.389 and cup volume (r = 0.491. Conclusion: Biochemically controlled acromegalic patients showed significantly higher CCT and IOP levels and lower RNFL thickness compared to healthy controls and the duration of disease was correlated with CCT and IOP levels.

  15. Growth Hormone and Endocrinopathies

    This is an analysis of 39 patients studied at the Yonsei Medical Center from January, 1976 to March 1979. Of these 35 patient were suspected of having hypothalamic insufficiency and subjected to the L-Dopa stimulation test to observe growth hormone secretary function while four acromegaly patient received the glucose loading test and L-Dopa stimulation test. The results are as follows: 1) The basal level of GH in the various disease was as follows: a) The basal level was lower than the control level but was not statistically significant b) In diabetes the mean value tended to higher than the control level but was not significant statistically c) In all four acromegaly patients the GH level was significantly higher than the control level 2) Of 13 patients with diabetes, nine had diabetic retinopathy, and of those nine, six showed increased L-Dopa response. However, of the four non retinopathic DM patients, only one showed increased response to L-Dopa. 3) Two patients out of ten with Sheehan's syndrome responded to L-Dopa stimulation. 4) One Patient of eight with pituitary chromophobe adenoma responded to L-Dopa stimulation. 5) Four acromegaly patients revealed 3 acidophilic adenoma and one chromophobe adenoma histologically. Of patients receiving the L-Dopa stimulation test. Two showed a paradoxical response. Two patients who received the glucose loading test showed suppressed response. 6) Of two craniopharyngioma patients, one showed increased GH response after L-Dopa stimulation. Increased response of GH after L-Dopa stimulation was seen in one two craniopharyngioma patients and also in one of two patients with short structure.

  16. Anaesthetic management for caesarean section in a case of previously operated with residual pituitary tumour

    Prerana N Shah

    2011-01-01

    Full Text Available Successful anaesthetic management for caesarean section in a case with previous pituitary tumour resection, with residual tumour, is reported. The pituitary gland undergoes global hyperplasia during pregnancy. Functional pituitary tumours may exhibit symptomatic enlargement during pregnancy. Growth hormone secreting tumour is associated with acromegaly which has associated anaesthetic implications of difficult airway, systemic hypertension, and diabetes and electrolyte imbalance. Intracranial space occupying lesions can increase intra cranial pressure and compromise cerebral perfusion or cause herniation. We report management of this case.

  17. Radioreceptor assay of human growth hormone

    A radioreceptor assay for human growth hormone (hGH) was developed. The receptor preparation was 25,000g pellet from the livers of pregnant rabbits. Iodination of GH with 125I was preformed by the methods of Lactoperoxidase and Iodogen. The sensitivity of assay was 0.67 ± 0.11 ng/ml serum. Serum hGH levels in 72 cases of normal subjects, 102 cases of acromegaly were measured by radioreceptor assay (RRA), and the results were compared with those obtained by radioimmunoassay (RIA)

  18. [Other specific types of diabetes].

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

    2016-04-01

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

  19. Transsphenoid surgery of pituitary adenoma of growth hormone%经蝶窦入路手术治疗垂体生长激素腺瘤

    高广忠; 卞留贯

    2011-01-01

    Objective To explore the effect of tumor size, preoperative GH and the intraoperative growth hormone ( GH) measurement on the operative outcome in pituitary adenoma of GH. Methods The clinical data of 47 patients with acromegaly wereanalyzed retrospectively . The oral glucose tolerance test was used in pre-operative diagnosis and post-operative evaluation. All the patients were operated through transsphenoid and the intraoperative GH was measured in 19 patients. Results After the operation, the symptoms of acromegaly were significantly relieved in 30 patients. According to the criteria of remission, there were 29 (61. 7% ) . However, the remission rate of microadenoma (77. 7%) was significantly higher than that in macroadenoma (51. 7%). Conclusion The early diagnosis of acromegaly is the crucial of the improvement of surgical outcome. For the doubt cases,OGTT is helpful for the early diagnosis. The transsphenoid approach is the first choice of the treatment of acromegaly. The intraoperative GH measurement is valuable for the early evaluation of surgical outcome. If the intraoperative GH can be rapidly and timely measured, it will be effective to increase the remission rate.%目的 探讨垂体生长激素(GH)腺瘤患者的肿瘤大小、术前GH水平、术中GH的动态变化对手术效果的影响.方法 回顾分析47例垂体GH腺瘤,18例术前行口服糖耐量试验(OGTT);均采用经蝶窦入路手术,19例术中检测GH水平,21例术后1个月行OGTT试验.结果 术后随访期内临床症状明显缓解的30例,随访期内GH<2.0.g/ml的有29例,总的缓解率61.7%,其中微腺瘤77.7%、大腺瘤的缓解率是51.7%.结论 垂体GH腺瘤患者的早期诊断是提高手术效果的关键,OGTT抑制试验有助于早期诊断;经鼻蝶入路手术是治疗首选,术中GH的动态观察有助于早期判断手术效果,若能及时快速测定GH,将有助于提高缓解率.

  20. Sequels after radiotherapy of adenoma of adenohypophysis. Folgen einer Hypophysenadenombestrahlung

    Knuepffer, J.; Helpap, B. (Staedtisches Krankenhaus, Singen (Germany). Inst. fuer Pathologie); Saeger, W. (Marienkrankenhaus, Hamburg (Germany). Abt. fuer Pathologie)

    1991-10-01

    14 years before his death, iridium seeds were implanted in the adenohypophysis of a 69-year-old patient with a STH producing adenoma of the adenohypophysis and acromegaly. 5 years after recurrence, transsphenoidal hypophysectomy was done. The patient died of central dysregulation due to cerebral insults. At autopsy, a recurrence of the undifferentiated acidophilic adenoma was found within the sphenoidal bone corpus. Immunohistochemistry was positive for STH, prolactin, TSH, LH, and FSH. Besides the adenoma, a mucoepidermoid carcinoma was found within the sphenoid cavity. This carcinoma may be a consequence of the radiotherapy of the adenoma of the adenohypophysis. (orig.).

  1. Sequels after radiotherapy of adenoma of adenohypophysis

    14 years before his death, iridium seeds were implanted in the adenohypophysis of a 69-year-old patient with a STH producing adenoma of the adenohypophysis and acromegaly. 5 years after recurrence, transsphenoidal hypophysectomy was done. The patient died of central dysregulation due to cerebral insults. At autopsy, a recurrence of the undifferentiated acidophilic adenoma was found within the sphenoidal bone corpus. Immunohistochemistry was positive for STH, prolactin, TSH, LH, and FSH. Besides the adenoma, a mucoepidermoid carcinoma was found within the sphenoid cavity. This carcinoma may be a consequence of the radiotherapy of the adenoma of the adenohypophysis. (orig.)

  2. 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

    Several in-vitro studies have suggested that the biological actions of IGF-I can be modified by the presence of specific IGF binding proteins. In man, the 24-hour serum levels of IGF-I and IGFBP-3 remain constant, but short-term changes in the IGF-I/IGFBP-3 ratio have been described following GH...... 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....

  3. Pregnancy and pituitary adenomas.

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

    2016-09-01

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

  4. In the land of giants: the legacy of José Dantas de Souza Leite

    Hélio A. G. Teive

    2015-07-01

    Full Text Available The authors describe the extraordinary contribution to science made by José Dantas de Souza Leite, who graduated from the Bahia School of Medicine and trained in Prof. Charcot’s Neurology Service under the supervision of Charcot’s most able pupil, Dr. Pierre Marie. Souza Leite presented his doctoral thesis on acromegaly, in Paris in 1890, and in the following year both him and Pierre Marie published a book on the subject, “Essays on Acromegaly”. This exceptional work established Souza Leite internationally as an important researcher, and the first Brazilian physician to contribute to the development of neuroendocrinology in an innovative way.

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

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

    2015-10-01

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

  6. Mortality in patients with pituitary disease.

    Sherlock, Mark

    2010-06-01

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

  7. Pregnancy and pituitary disorders: Challenges in diagnosis and management

    Bashir A Laway

    2013-01-01

    Full Text Available Pregnancy is associated with normal physiological changes in endocrine system that assists fetal survival as well as preparation of labor. The pituitary gland is one of the most affected organs in which major changes in anatomy and physiology take place. Due to overlapping clinical and biochemical features of pregnancy, sometimes the diagnosis of pituitary disorders may be challenging. It is important to know what normal parameters of changes occur in endocrine system in order to diagnose and manage complex endocrine problems in pregnancy. In our present review, we will focus on pituitary disorders that occur exclusively during pregnancy like Sheehan′s syndrome and lymphocytic hypophysitis and pre-existing pituitary disorders (like prolactinoma, Cushing′s disease and acromegaly, which poses significant challenge to endocrinologists.

  8. Pseudoacromegaly in congenital generalised lipodystrophy (Berardinelli-Seip syndrome).

    Chakraborty, Partha Pratim; Datta, Saumik; Mukhopadhyay, Satinath; Chowdhury, Subhankar

    2016-01-01

    Pseudoacromegaly, or acromegaloidism, is characterised by a clinical appearance mimicking acromegaly in the absence of documented hypersomatotropism or past exposure to excess growth hormone. It can develop secondary to a number of congenital and acquired conditions of which severe insulin resistance is an important example. Lipodystrophy syndromes are a group of rare disorders of which autosomal recessive congenital generalised lipodystrophy is the most common type. Patients with this disorder are predisposed to insulin resistance and its associated complications such as diabetes mellitus, hypertriglyceridaemia, fatty liver, polycystic ovaries and acanthosis nigricans. Elevated circulating insulin levels in these patients rarely can give rise to soft tissue and bony overgrowth, with resultant acromegaloidism. We report an adolescent girl presenting with unusual prominence of her hands and feet; a thorough evaluation ultimately revealed a diagnosis of congenital generalised lipodystrophy. PMID:27068725

  9. Radioimmunoassay of human growth hormone and its application in pituitary dysfunction studies

    A simple, specific and sensitive Radioimmunoassay (RIA) has been developed for the measurement of Human Growth Hormone (HGH) in serum samples. 123I-labelled HGH has been used as a tracer and dextran coated charcoal system has been employed to separate antibody bound hormone from the unbound one. The assay offers sensitivity of 0.16 ng/ml with a reproducibility of 7% intraassay and inter-assay variations. Serum HGH levels were measured at fasting-resting state and during insulin stimulation test in (1) 15 normal subjects (controls) and (2) 31 patients with stunted growth, whereas (3) in 7 acromegalic patients the same were measured at fasting-resting state and after oral glucose administration. This procedure has been used to distinguish dwarfs due to growth hormone deficiency from other conditions unrelated to pituitary disease and to confirm acromegaly. (author)

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

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

    1992-10-01

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

  11. Magnetic resonance imaging of pituitary adenomas

    Bonneville, Jean-Francois; Bonneville, Fabrice; Cattin, Francoise [University Hospital, Department of Neuroradiology, Besancon (France)

    2005-03-01

    Today, MR is the only method needed for the morphological investigation of endocrine-active pituitary adenomas. In acromegaly and Cushing's syndrome, the therapeutic attitude is directly dictated by MR data. We present the MR aspect of pituitary adenomas according to size, sex, age, endocrine activity and a few particular conditions such as hemorrhagic pituitary adenomas, pituitary adenomas during pregnancy, cavernous sinus invasion and postsurgical changes. When an intrasellar mass extending out of the sella turcica is detected, the goal of the MR examination is to indicate precisely the origin of the tumor, its extension in relation to the various surrounding structures, its structure and its enhancement in order to help in the differential diagnosis. Demonstration of very small pituitary adenomas remains a challenge. When SE T1- and Turbo SE T2-weighted sequences are non-diagnostic, enhanced imaging becomes mandatory; half-dose gadolinium injection, delayed sequence, dynamic imaging can be of some help. (orig.)

  12. 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...... of OCT on the IGF system could have therapeutical implications in diabetes mellitus, cardiovascular disease, and certain malignancies in which IGF-I might be involved. The aim of this study was to examine possible GH-independent effects of OCT on IGF components in humans. Six GH-deficient (GHD......) patients were studied for 24 h after each of the following treatment regimens (each of 1 weeks duration): (a) daily s.c. GH injection (2 IU/m(2)); (b) as (a) + continuous s.c. infusion of OCT (200 microg/24 h) by means of a portable pump (Nordic Infuser); (c) no treatment. Serum GH binding protein (GHBP...

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

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

  14. Immunohistochemical detection of dopamine D2 receptors in human pituitary adenomas.

    Pawlikowski, Marek

    2010-09-30

    Thirty one pituitary adenomas and 3 samples of peritumoral anterior pituitary tissue were immunostained with an antibody raised against dopamine D2 receptor protein. The positive reactions were found in cell cytoplasm, a subpopulation of cell nuclei and the intratumoral blood vessels walls. As expected, the positive immunostaining was shown in cytoplasm and/or cell nuclei of all examined prolactinomas (7/7). In acromegaly the positive D2 staining occurred in 5/7 samples, in gonadotropinomas in 6/8 and in plurihormonal adenomas 2/4. The lowest expression was observed in corticotropinomas (1/5). These findings corroborate with the well known efficacy of D2 agonists in the treatment of prolactinomas and somatotropinomas, and support the rationale of the therapeutic trials with these compounds in gonadotropinomas. Moreover, the presence of D2 receptors in intratumoral blood vessels walls constitutes the possibility of the anti-angiogenic action of D2 agonists in pituitary adenomas. PMID:21071344

  15. Immunohistochemical detection of dopamine D2 receptors in human pituitary adenomas.

    Marek Pawlikowski

    2010-11-01

    Full Text Available Thirty one pituitary adenomas and 3 samples of peritumoral anterior pituitary tissue were immunostained with an antibody raised against dopamine D2 receptor protein. The positive reactions were found in cell cytoplasm, a subpopulation of cell nuclei and the intratumoral blood vessels walls. As expected, the positive immunostaining was shown in cytoplasm and/or cell nuclei of all examined prolactinomas (7/7. In acromegaly the positive D2 staining occurred in 5/7 samples, in gonadotropinomas in 6/8 and in plurihormonal adenomas 2/4. The lowest expression was observed in corticotropinomas (1/5. These findings corroborate with the well known efficacy of D2 agonists in the treatment of prolactinomas and somatotropinomas, and support the rationale of the therapeutic trials with these compounds in gonadotropinomas. Moreover, the presence of D2 receptors in intratumoral blood vessels walls constitutes the possibility of the anti-angiogenic action of D2 agonists in pituitary adenomas.

  16. [Endocrine and other medical causes of abnormal fatigability].

    Bürgi, U

    1991-11-01

    Many endocrine diseases can cause fatigue. Tiredness is a frequent symptom of primary and secondary hypothyroidism, hyperthyroidism, excessive glucocorticoid or mineralocorticoid production, primary and secondary adrenal insufficiency, primary and secondary hypogonadism and hyperprolactinemia in the male, acromegaly, diabetes mellitus and diabetes insipidus. A great number of medical diseases other than those mentioned in the articles on cardiological and pneumological fatigue can also cause abnormal tiredness (infectious diseases, hematological, renal, hepatic, gastrointestinal and rheumatological disturbances, vasculitis and malignant tumors). The pathogenesis of tiredness caused by endocrine or medical illnesses, i.e. how the sensation of fatigue is produced, is not clear. The fatigue of the various endocrine or other medical diseases is not disease-specific, i.e. its characteristics do not differentiate it from the fatigue of other illnesses. PMID:1754971

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

    Bastan Hagh M

    1997-07-01

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

  18. Pituitary macroadenomas: reviews of 60 cases; Revisao de 60 casos de macroadenomas hipofisarios

    Santos, Alair Augusto S.M.D. dos; Fontes, Cristina Asvolinsque P.; Magnago, Marcelo [Universidade Federal Fluminense, Niteroi, RJ (Brazil). Inst. de Radiologia; Moreira, Denise Madeira [Universidade Federal, Rio de Janeiro, RJ (Brazil). Inst. de Radiologia; Andreiuolo, Pedro Angelo [Hospital Santa Cruz/Beneficencia Portuguesa, Niteroi, RJ (Brazil). Servico de Radiologia; Oliveira, Fernando Barros de; Teixeira, Ricardo Tostes D.; Correa, Saul Orlando C. [Universidade Federal, Rio de Janeiro, RJ (Brazil). Inst. de Biofisica Carlos Chagas Filho. Curso de Pos-graduacao Medica

    2000-12-01

    We reviewed 60 cases of patients with pituitary macroadenomas who were submitted to magnetic resonance imaging evaluations at private institutions in Niteroi and Rio de Janeiro, Brazil. The aim of this study was to demonstrate the advantages of magnetic resonance imaging in the diagnosis and follow-up of these patients. Macroprolactinomas were the most prevalent tumors. We also observed four growth-hormone (GH) secreting macroadenomas with clinical signs of acromegaly, and one GH- and prolactin-secreting macroadenoma. Seven patients presented pituitary apoplexy and hyperintense signal on T1-W images before contrast medium (gadolinium) administration suggestive of intratumoral bleeding. The follow-up of 15 cases demonstrated a reduction in the size of the tumor after surgery, clinical treatment or radiotherapy. Magnetic resonance imaging is important for the study of pituitary macroadenomas, particularly before surgery, as it shows the involvement of adjacent structures, specially the cavernous sinus, optic chiasm and pituitary stalk compression. (author)

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

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

    1989-12-01

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

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

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

    1989-12-01

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

  1. Pituitary macroadenomas: reviews of 60 cases

    We reviewed 60 cases of patients with pituitary macroadenomas who were submitted to magnetic resonance imaging evaluations at private institutions in Niteroi and Rio de Janeiro, Brazil. The aim of this study was to demonstrate the advantages of magnetic resonance imaging in the diagnosis and follow-up of these patients. Macroprolactinomas were the most prevalent tumors. We also observed four growth-hormone (GH) secreting macroadenomas with clinical signs of acromegaly, and one GH- and prolactin-secreting macroadenoma. Seven patients presented pituitary apoplexy and hyperintense signal on T1-W images before contrast medium (gadolinium) administration suggestive of intratumoral bleeding. The follow-up of 15 cases demonstrated a reduction in the size of the tumor after surgery, clinical treatment or radiotherapy. Magnetic resonance imaging is important for the study of pituitary macroadenomas, particularly before surgery, as it shows the involvement of adjacent structures, specially the cavernous sinus, optic chiasm and pituitary stalk compression. (author)

  2. Validation of somatostatin receptor scintigraphy in the localization of neuroendocrine tumors

    Somatostatin analogs are used in the control of hormonal hypersecretion and tumor growth of patients with acromegaly, islet cell carcinomas and carcinoids. Recently we showed that somatostatin receptor positive tumors can be visualized in vivo after the administration of radionuclide-labeled somatostatin analogs. Receptor imaging was positive in 18/21 islet cell tumors, 32/37 carcinoids, 26/28 paragangliomas, 9/14 medullary thyroid carcinomas, and 5/7 small cell lung cancers. Somatostatin receptor imaging is an easy, harmless and painless diagnostic method. It localizes multiple and/or metastatic tumors, predicts the successful control of hormonal hypersecretion by octreotide and seems to be of prognostic value in certain types of cancer. This scintigraphic method might help in patient selection for clinical trials with somatostatin analogs in the treatment of neuroendocrine cancers. (orig.)

  3. Berardinelli-Seip syndrome: highlight of treatment challenge.

    Ferraria, Nélia; Pedrosa, Cristina; Amaral, Daniela; Lopes, Lurdes

    2013-01-01

    Berardinelli-Seip congenital lipodystrophy (BSCL) syndrome is a rare autosomal-recessive disease characterised by lipoatrophy and associated with deregulations of glycidic and lipid metabolism. We report three BSCL cases with its typical clinical picture and complications. Clinically, they all show marked atrophy of adipose tissue, acromegaly, acanthosis nigricans and tall stature. Two cases present attention deficit hyperactivity and developmental learning disorders; another patient has hypertrophic myocardiopathy and polycystic ovary syndrome. In all the cases AGPAT2 was the identified mutation. All the cases present hypertriglyceridemia. One case has developed hyperinsulinism controlled with metformin and another case already has type 2 diabetes with a difficult clinical control. There is no curative treatment and the current treatment options are based only on symptomatic control of the complications. Recently, published studies showed that leptin-replacement therapy appears a promising tool in the metabolic correction of BSCL complications, highlighting the importance of further investigations in BSCL treatment. PMID:23362058

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

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

  5. Attenuated RORC expression in the presence of EMT progression in somatotroph adenomas following treatment with somatostatin analogs is associated with poor clinical recovery.

    Tove Lekva

    Full Text Available Somatostatin analogs (SA have been established as the first line medical treatment for acromegaly, but following long-term treatment, SA normalizes GH and IGF-I levels in only 40-60% of patients. The epithelial marker E-cadherin plays a crucial role in the epithelial mesenchymal transition (EMT and is associated with a poor response to SA treatment. We hypothesized that the characterization of transcripts regulated by SA in somatotroph adenomas with high and low E-cadherin expression may identify signaling pathways and mediators that can explain the poor response to SA treatment. We performed a microarray analysis of sixteen adenomas with different levels of E-cadherin and SA treatment to identify regulated transcripts. Candidate transcripts were further explored in vivo in sixty-five adenomas, and interactions between SA treatment and EMT progression on mRNA expression profiles and associations with clinical recovery were assessed. Finally, the effects of SA treatment on adenoma cells in vitro from acromegalic patients were determined. Microarray analysis of selected adenomas with differential E-cadherin expression, as a marker of EMT progression, identified 172 genes that displayed differential expression that was dependent on SA treatment. The validation of selected candidates in the entire cohort identified 9 transcripts that showed an interaction between E-cadherin expression and SA treatment. Further analysis of the impact of these genes suggests that attenuated RORC expression in somatotroph adenomas is associated with increased tumor size and a blunted clinical response. Our study indicates that attenuated RORC may be involved in the poor clinical response to SA treatment in patients with acromegaly.

  6. Evaluation of the increase in GH and IGF-1 and effectiveness in the treatment on Zacatecas population; Evaluacion del aumento en GH e IGF-1 y eficacia en el tratamiento en poblacion zacatecana

    Gallegos F, P. I.; Badillo A, V., E-mail: perla_gf17@hotmail.com [Universidad Autonoma de Zacatecas, Unidad Academica de Estudios Nucleares, Laboratorio de Radioinmunoanalisis y Quimioluminiscencia, Cipres No. 10, Fracc. La Penuela, 98060 Zacatecas (Mexico)

    2013-10-15

    The acromegaly and gigantism are dysfunctions that are caused by hyper-secretion of growth hormone (GH) and of production in liver of growth factor similar to the insulin type 1 (IGF-1) mediated by the GH secretion. The secretor pituitary adenomas of GH are the main cause of the hyper-secretion. The acromegaly and gigantism are manifested respectively by acral alterations and extremities increase, and an excessive growth of the bones. Although a world prevalence of 40-60 cases by inhabitants million is registered, very few formal studies exist that confirm this number. According to the program Epiacro in Mexico is considered a prevalence of 13 cases by inhabitants million. In the Zacatecas State official statistical numbers are not had for these pathologies. Due to the few registrations that exist, or to the cases reported in Mexico, is necessary to evaluate patients with suspicion and with hyper-secretion diagnostic of GH, to contribute and/or to reinforce the health state and national statistics. In this work the GH and IGF-1 concentrations were measured on Zacatecas population to estimate the age range and sex with more probability of suffering this illness, and to evaluate the patients that have received some treatment to check their effectiveness verifying the GH and IGF-1 decrease and being able to obtain normal values. We register 26 patient cases with suspicion of GH hyper-secretion, of these 9 were affected by the illness. The hyper-secretion cases were presented with more frequency in half age adults, being affected in a same way as much men as women. To the end of the study only an affected patient concludes with the pharmacological treatment for the GH hyper-secretion control of a group of 5. (Author)

  7. Growth hormone-secreting macroadenoma of the pituitary gland successfully treated with the radiolabeled somatostatin analog (90)Y-DOTATATE: case report.

    Waligórska-Stachura, Joanna; Gut, Paweł; Sawicka-Gutaj, Nadia; Liebert, Włodzimierz; Gryczyńska, Maria; Baszko-Błaszyk, Daria; Blanco-Gangoo, Al Ricardo; Ruchała, Marek

    2016-08-01

    Pituitary tumors causing acromegaly are usually macroadenomas at the time of diagnosis, and they can grow aggressively, infiltrating surrounding tissues. Difficulty in achieving complete tumor removal at surgery can lead toward a strong tendency for recurrence, making it necessary to consider a means of treatment other than those currently used such as somatostatin analogs (SSAs), growth hormone (GH) receptor antagonist, surgical removal, and radiotherapy. The purpose of this paper is to describe a patient diagnosed with an aggressive, giant GH-secreting tumor refractory to medical therapy but ultimately treated with the radiolabeled somatostatin analog (90)Y-DOTATATE. A 26-year-old male with an invasive macroadenoma of the pituitary gland (5.6 × 2.5 × 3.6 cm) and biochemically confirmed acromegaly underwent 2 partial tumor resections: the first used the transsphenoidal approach and the second used the transcranial method. The patient received SSAs pre- and postoperatively. Because of the progression in pituitary tumor size, he underwent classic irradiation of the tumor (50 Gy). One and a half years later, the patient presented with clinically and biochemically active disease, and the tumor size was still 52 mm in diameter (height). Two neurosurgeons disqualified him from further surgical procedures. After confirming the presence of somatostatin receptors in the pituitary tumor by using (68)Ga-DOTATATE PET/CT, we treated the patient 4 times with an SSA bound with (90)Y-DOTATATE. After this treatment, the patient attained partial biochemical remission and a reduction in the tumor mass for the first time. Treatment with an SSA bound with (90)Y-DOTATATE may be a promising option for some aggressive GH-secreting pituitary adenomas when other methods have failed. PMID:26636388

  8. Comparison of acid ethanol extraction and acid gel filtration prior to IGF-I and IGF-II radioimmunoassays; Improvement of determinations in acid ethanol extracts by the use of truncated IGF-I as radioligand

    Bang, P.; Eriksson, U.; Wivall, I.-L.; Hall, K. (Department of Endocrinology, Karolinska Institute, Stockholm (Sweden)); Sara, V. (Department of Pathology, Karolinska Institute, Stockholm (Sweden))

    1991-01-01

    Insulin-like growth factor binding proteins interfere in the IGF-I and -II radioimmunoassays. In an attempt to overcome this problem, we have compared the use of truncated IGF-I, with reduced IGFBP affinity, and IGF-I as radioligands for IGF-I RIA measurements in serum separated by acid gel filtration or acid ethanol extraction followed by cryo-precipitation. With truncated IGF-I as radioligand the IGF-I measurements in acid gel filtrates and acid ethanol extracts were significantly correlated in healthy subjects (N=42, r=0.91, p<0.001) and in patients with acromegaly (N=10, r=0.85, p<0.01), GH deficiency (N=10, r=0.88, p<0.001) or Type I diabetes mellitus (N=10, r=0.90, p<0.001). In contrast, the IGF-I concentrations in acid ethanol extracts determined with IGF-I as radioligand did not correlate with those in acid gel filtrates using truncated IGF-I radioligand in patients with acromegaly (r=0.61, NS) or GH deficiency (r=0.46, NS). In the latter group the mean IGF-I concentrations measured in acid ethanol extracts were erroneously elevated by 112%. Low-affinity antibodies used for IGF-II RIA determinations failed to give reliable results in acid ethanol extracts from patients with Type I diabetes mellitus or GH deficiency. In conclusion, erroneously high IGF-I concentrations owing to binding of the radioligand to IGFBPs not completely removed by acid ethanol extraction can be avoided by the use of truncated IGF-I as radioligand. (author).

  9. Locally produced estrogen through aromatization might enhance tissue expression of pituitary tumor transforming gene and fibroblast growth factor 2 in growth hormone-secreting adenomas.

    Ozkaya, Hande Mefkure; Comunoglu, Nil; Keskin, Fatma Ela; Oz, Buge; Haliloglu, Ozlem Asmaz; Tanriover, Necmettin; Gazioglu, Nurperi; Kadioglu, Pinar

    2016-06-01

    Aromatase, a key enzyme in local estrogen synthesis, is expressed in different pituitary tumors including growth hormone (GH)-secreting adenomas. We aimed to evaluate aromatase, estrogen receptor α (ERα), estrogen receptor β (ERβ), pituitary tumor transforming gene (PTTG), and fibroblast growth factor 2 (FGF2) expressions in GH-secreting adenomas, and investigate their correlation with clinical, pathologic, and radiologic parameters. This cross-sectional study was conducted in a tertiary center in Turkey. Protein expressions were determined via immunohistochemical staining in ex vivo tumor samples of 62 patients with acromegaly and ten normal pituitary tissues. Concordantly increased aromatase, PTTG, and FGF2 expressions were detected in the tumor samples as compared with controls (p tumors expressed ERα while ERβ was detected only in mixed somatotroph adenomas. Aromatase, ERβ, PTTG expressions were not significantly different between patients with and without remission (p > 0.05 for all). FGF2 expression was significantly higher in patients without postoperative and late remission (p = 0.002 and p = 0.012, respectively), with sphenoid bone invasion, optic chiasm compression, and somatostatin analog resistance (p = 0.005, p = 0.033, and p = 0.013, respectively). Aromatase, PTTG and FGF2 expressions were positively correlated with each other (r = 0,311, p = 0.008 for aromatase, FGF2; r = 0.380, p = 0.001 for aromatase, PTTG; r = 0.400, p = 0.001 for FGF2, PTTG). PTTG-mediated FGF2 upregulation is associated with more aggressive tumor features in patients with acromegaly. Also, locally produced estrogen through aromatization might have a role in this phenomenon. PMID:26578364

  10. Evaluation of the increase in GH and IGF-1 and effectiveness in the treatment on Zacatecas population

    The acromegaly and gigantism are dysfunctions that are caused by hyper-secretion of growth hormone (GH) and of production in liver of growth factor similar to the insulin type 1 (IGF-1) mediated by the GH secretion. The secretor pituitary adenomas of GH are the main cause of the hyper-secretion. The acromegaly and gigantism are manifested respectively by acral alterations and extremities increase, and an excessive growth of the bones. Although a world prevalence of 40-60 cases by inhabitants million is registered, very few formal studies exist that confirm this number. According to the program Epiacro in Mexico is considered a prevalence of 13 cases by inhabitants million. In the Zacatecas State official statistical numbers are not had for these pathologies. Due to the few registrations that exist, or to the cases reported in Mexico, is necessary to evaluate patients with suspicion and with hyper-secretion diagnostic of GH, to contribute and/or to reinforce the health state and national statistics. In this work the GH and IGF-1 concentrations were measured on Zacatecas population to estimate the age range and sex with more probability of suffering this illness, and to evaluate the patients that have received some treatment to check their effectiveness verifying the GH and IGF-1 decrease and being able to obtain normal values. We register 26 patient cases with suspicion of GH hyper-secretion, of these 9 were affected by the illness. The hyper-secretion cases were presented with more frequency in half age adults, being affected in a same way as much men as women. To the end of the study only an affected patient concludes with the pharmacological treatment for the GH hyper-secretion control of a group of 5. (Author)

  11. Rheumatoid Arthritis Associated with the Use of Sandostatin® LAR® Depot in a Patient with Pancreatic Neuroendocrine Tumor. An Association or a Coincidence? The First Case Report

    Muhammad Wasif Saif

    2011-07-01

    Full Text Available Context Sandostatin® LAR® depot is a synthetic analogue of the naturally occurring hormone somatostatin and is indicated for certain patients with acromegaly and severe diarrhea and flushing episodes associated with metastatic carcinoid tumors and for the long-term treatment of the profuse watery diarrhea associated with VIP-secreting tumors in patients in whom initial treatment with immediate release Sandostatin® injection has been shown to be effective and tolerated. The most common toxicities include biliary disorders, gastrointestinal disorders, injection-site pain, hypoglycemia and hyperglycemia. Rheumatoid arthritis or similar toxicities have not been associated with Sandostatin® LAR® depot. Case report We present a 53-year-old female with a history of neuroendocrine tumor of the pancreas with metastasis to the liver, lung developed joint pains in the hands as well as feet accompanied with intermittent swelling in the morning and pain in the bilateral joints in the hands as well as feet following 45th cycle of Sandostatin® LAR® depot at a dose of 30 mg. All the work-up including rheumatoid factor, anti nuclear antibody, cryoglobulins were within normal limits except her erythrocyte sedimentation rate was elevated. No radiological abnormalities were revealed. Her symptoms improved after we reduced the dose to 20 mg. Discussion Her Naranjo scale was 7, suggesting a probable relation. The patient had four signs and symptoms as required by the American College of Rheumatology for the diagnosis of rheumatoid arthritis. The association of the rheumatoid arthritis with Sandostatin® LAR® depot may be a rare complication but with the extended use beyond acromegaly and carcinoid to acute esophageal variceal bleeding, pancreatic pseudocysts, gastrointestinal, and pancreatic external fistulae, short bowel syndrome, a dumping syndrome and acquired immunodeficiency syndrome-related refractory hypersecretory diarrhea, physicians should be made

  12. Avaliação plasmática de igf-1 no prolactinoma IGF-1 plasma levels evaluation in prolactinoma

    Daniela Zylberberg

    2006-09-01

    Full Text Available Prolactinomas são os tumores hipofisários mais comuns, podendo co-secretar GH (hormônio do crescimento. IGF-1 (fator de crescimento insulina-símile-1 é o principal responsável pelas ações do GH e parâmetro diagnóstico de acromegalia. Objetivando determinar por uma dosagem de IGF-1, na avaliação inicial de pacientes com prolactinoma, ocorrência de tumores mistos [GH e prolactina (PRL], estudamos 7 homens e 27 mulheres, entre 19 e 72 anos, confrontando-os aos resultados de GH basal e durante teste oral de tolerância à glicose, quando GH basal >0,4 ng/mL ou níveis de IGF-1 alterados. A proporção de pacientes com GH >0,4 ng/mL e IGF-1 elevada foi alta; mas, após administração de 75g de glicose por via oral, nenhum paciente foi diagnosticado como acromegálico. Sugerimos, porém que a dosagem de IGF-1 seja realizada pelo risco de co-secreção de GH nos prolactinomas. Atenção especial para pacientes que apresentem significativa diminuição dos níveis de PRL, sem correspondente regressão do tamanho do adenoma.Prolactinomas are the most frequent pituitary tumors and may co-secrete GH (growth hormone. IGF-1 (insulin-like growth factor-1 is the main responsible for GH actions and a parameter for the diagnosis of acromegaly. With the objective of identifying through a IGF-1 levels analysis, in the initial evaluation of prolactinoma patients, the existence of mixed tumors [GH and prolactin (PRL], we studied 7 men and 27 women, aged between 19 and 72 years, confronting them with the results of basal and glucose stimulated (glucose tolerance test - GTT GH levels, indicated when GH >0.4 ng/mL or IGF-1 levels were elevated. The prevalence of patients with GH >0.4 ng/mL and elevated IGF-1 was higher than expected; however, after GTT, no patient fulfilled the diagnostic criteria for acromegaly. However, we suggest that, they should be submitted to IGF-1 evaluation, due to the risk of GH co-secretion in prolactinomas. Special attention

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

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

    2011-09-15

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

  14. Tc99m-Tetrofosmin and Thallium-201 SPECT imaging of pituitary tumors: Preliminary results

    Aim: It has been reported that pituitary adenomas accumulate Thallium -201(Tl-201), Tc99m-sestamibi and In111-Octreotide. These agents maybe useful in the diagnosis and follow-up of pituitary adenomas. Tc99m-tetrofosmin also has been shown as an tumor seeking agent in various tumors. The aim of this study is to evaluate the accumulation of Tc99m-tetrofosmin in pituitary adenomas and compare the results to Tl-201 SPECT. Methods: We performed Tc99m-Tetrofosmin and Tl-201 SPECT imaging on 5 patients (pts) with pituitary tumors, 3 patients with acromegaly, 1 patient with macro prolactinoma, and 1 patient with nonfunctional tumor (1 female, 4 male, age range: 26-50). SPECT imaging of cranium were obtained 20 min after intravenous injection of 111 MBq Tl-201 and 555 MBq Tc99m-Tetrofosmin on different days with a dual head gamma camera. All but one patient had surgical therapy. One patient had medical treatment. One patient had post surgical imaging with both Tetrofosmin and Tl-201 and 2 pts had only with Thallium. Semiquantitative analysis were performed by calculating tumor to background ratio from the mean counts of the created ROI's. Results: All adenomas showed prominent uptake with both Thallium and Tetrofosmin. The mean tumor to background ratios were 2.3 (range: 1.2-4.29) with Thallium and 3.57 (range: 1.6-6.86) with Tetrofosmin. Post therapy images showed no significant uptake in pituitary region with both agents in 2 patients. Post therapy mean Thallium uptake ratio in 3 pts was 1.64. One patient with acromegaly showed no decrease in thallium uptake, but showed some decrease in tetrofosmin uptake. Conclusion: Although our patient number is limited, these preliminary results indicate that Tc99m-tetrofosmin has also strong affinity for pituitary adenomas as does Thallium, either the adenoma is functioning or non-functioning. It may have a potential value to evaluate the response to therapy

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

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

  16. Silent subtype 3 pituitary adenomas are not always silent and represent poorly differentiated monomorphous plurihormonal Pit-1 lineage adenomas.

    Mete, Ozgur; Gomez-Hernandez, Karen; Kucharczyk, Walter; Ridout, Rowena; Zadeh, Gelareh; Gentili, Fred; Ezzat, Shereen; Asa, Sylvia L

    2016-02-01

    Originally classified as a variant of silent corticotroph adenoma, silent subtype 3 adenomas are a distinct histologic variant of pituitary adenoma of unknown cytogenesis. We reviewed the clinical, biochemical, radiological, immunohistochemical and ultrastructural features of 31 silent subtype 3 adenomas to clarify their cellular origin. Among 25 with clinical and/or radiological data, all were macroadenomas; there was cavernous sinus invasion in 30% of cases and involvement of the clivus in 17% of cases. Almost 90% of patients were symptomatic; 67% had mass effect symptoms, 37% were hypogonadal and 8% had secondary adrenal insufficiency. Significant hormonal excess in 29% of cases included hyperthyroidism in 17%, acromegaly in 8% and hyperprolactinemia above 150 μg/l in 4%. Two individuals with hyperprolactinemia who were younger than 30 years had multiple endocrine neoplasia type 1. Immunohistochemically, all 31 tumors were diffusely positive for the pituitary lineage-specific transcription factor Pit-1. Although three only expressed Pit-1, others revealed variable positivity for one or more hormones of Pit-1 cell lineage (growth hormone, prolactin, thyroid-stimulating hormone), as well as alpha-subunit and estrogen receptor. Most tumors exhibited perinuclear reactivity for keratins with the CAM5.2 antibody; scattered fibrous bodies were noted in five (16%) tumors. The mean MIB-1 labeling index was 4% (range, 1-9%). Fourteen cases examined by electron microscopy were composed of a monomorphous population of large polygonal or elongated cells with nuclear spheridia. Sixty-five percent of patients had residual disease after surgery; after a mean follow-up of 48.4 months (median 41.5; range=2-171) disease progression was documented in 53% of those cases. These data identify silent subtype 3 adenomas as aggressive monomorphous plurihormonal adenomas of Pit-1 lineage that may be associated with hyperthyroidism, acromegaly or galactorrhea and amenorrhea. Our

  17. Clinical applications of somatostatin analogs for growth hormone-secreting pituitary adenomas

    Wang JW

    2014-01-01

    Full Text Available Ji-wen Wang,1,2 Ying Li,3 Zhi-gang Mao,1,2 Bin Hu,1,2 Xiao-bing Jiang,1,2 Bing-bing Song,4 Xin Wang,4 Yong-hong Zhu,4 Hai-jun Wang1,21Department of Neurosurgery and Pituitary Tumor Center, The First Affiliated Hospital, Sun Yat-sen University, 2Key Laboratory of Pituitary Adenoma in Guangdong Province, 3State Key Laboratory of Ophthalmology, Zhongshan Ophthalmic Center, Sun Yat-sen University, 4Department of Histology and Embryology, Zhongshan School of Medicine, Sun Yat-sen University, Guangzhou, People's Republic of ChinaAbstract: Excessive growth hormone (GH is usually secreted by GH-secreting pituitary adenomas and causes gigantism in juveniles or acromegaly in adults. The clinical complications involving cardiovascular, respiratory, and metabolic systems lead to elevated morbidity in acromegaly. Control of serum GH and insulin-like growth factor (IGF 1 hypersecretion by surgery or pharmacotherapy can decrease morbidity. Current pharmacotherapy includes somatostatin analogs (SAs and GH receptor antagonist; the former consists of lanreotide Autogel (ATG and octreotide long-acting release (LAR, and the latter refers to pegvisomant. As primary medical therapy, lanreotide ATG and octreotide LAR can be supplied in a long-lasting formulation to achieve biochemical control of GH and IGF-1 by subcutaneous injection every 4–6 weeks. Lanreotide ATG and octreotide LAR provide an effective medical treatment, whether as a primary or secondary therapy, for the treatment of GH-secreting pituitary adenoma; however, to maximize benefits with the least cost, several points should be emphasized before the application of SAs. A comprehensive assessment, especially of the observation of clinical predictors and preselection of SA treatment, should be completed in advance. A treatment process lasting at least 3 months should be implemented to achieve a long-term stable blood concentration. More satisfactory surgical outcomes for noninvasive macroadenomas treated

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

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

    1988-12-01

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

  19. Tumors in the region of the sella turcica; Tumoren der Sellaregion

    Reith, W. [Universitaetsklinikum des Saarlandes, Klinik fuer Diagnostischeund Interventionelle Neuroradiologie, Homburg/Saar (Germany)

    2009-07-15

    Tumors of the pituitary gland can lead to limitation of hypophysis function (hypophysis insufficiency) or hypersecretion of different hormones (acromegaly, Cushing's syndrome, prolactinoma, TSH-secreting adenoma). The optic chiasma lies in close proximity to the pituitary gland and can be compressed by tumors leading to visual disturbances (bilateral hemianopsia). Tumors can be separated into hormone secreting and hormone inactive tumors, as well as into microadenoma with a diameter <10 mm and macroadenomas >10 mm. A rare group of tumors of the hypophysis region are craniopharyngiomas, meningiomas, germinomas, gliomas, metastases and granulomotous inflammations, such as sarcoidosis and tuberculosis. (orig.) [German] Bei Tumoren der Hypophysenregion kann es zu einer Einschraenkung der Hypophysenfunktion (Hypophyseninsuffizienz) kommen, aber auch zu einem Hypersekretionssyndrom (Akromegalie, Cushing-Syndrom, Prolaktinom, TSH-sezernierendes Adenom). Unmittelbar ueber der Hypophysenregion liegt das Chiasma opticum, wodurch es bei Tumoren der Hypophysenregion zur Beeintraechtigung des Sehens kommen kann, meist als Verschlechterung des Sehfeldes beider Augen nach lateral (bitemporale Hemianopsie). Man unterscheidet hormonaktive und -inaktive Tumoren. Es werden Mikroadenome mit einem Durchmesser bis 10 mm und Makroadenome, die groesser als 10 mm sind, unterschieden. Eine seltenere Gruppe von Tumoren der Hypophysenregion sind die Kraniopharyngeome, Meningeome, Germinome, Gliome, Metastasen und granulomatoese Entzuendungen (Sarkoidose, Tuberkulose). (orig.)

  20. The effects of pituitary and thyroid disorders on haemostasis: potential clinical implications.

    Kyriakakis, Nikolaos; Lynch, Julie; Ajjan, Ramzi; Murray, Robert D

    2016-04-01

    Disturbances of coagulation and fibrinolysis are usually multifactorial and growing evidence suggests that endocrinopathies modulate the haemostatic balance. The thrombotic alterations in endocrine disorders range from mild laboratory clotting abnormalities with little clinical significance to serious thrombotic and bleeding disorders directly related to hormonal disturbances. This literature review focuses on presenting the current data on the effects of thyroid and pituitary disorders on various parameters of the haemostatic system. With the exception of overt hypothyroidism which appears to cause a bleeding tendency, the rest of the endocrinopathies discussed in this review (subclinical hypothyroidism, hyperthyroidism, endogenous hypercortisolaemia, growth hormone deficiency, acromegaly, prolactinoma/hyperprolactinaemia and hypogonadotrophic hypogonadism) are associated with a hypercoagulable and hypofibrinolytic state, increasing the overall cardiovascular risk and thromboembolic potential in these patients. In most studies, the haemostatic abnormalities seen in endocrine disorders are usually reversible with successful treatment of the underlying condition and biochemical disease remission. High-quality studies on larger patient cohorts are needed to produce robust evidence on the effects of endocrine disorders and their therapeutic interventions on coagulation and fibrinolysis, as well as on the long-term mortality and morbidity outcomes in association with endocrine-related haemostatic imbalance. Given the rarity of some of the endocrine disorders, multicentre studies are required to achieve this target. PMID:25753252

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

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

  2. Study of NSILA-s (nonsuppressible insulin-like activity soluble in acid ethanol) by a new radio-receptor assay

    The insulin-like activity nonsuppressible with insulin-antibodies (NSILA) accounts for 90% of the insulin activity of the blood plasma. A peptid, soluble in acid ethanol, was purified (NSILA-s) and specific NSILA-s receptors were found on the plasma membrane of liver cells. The specificity, kinetics, affinity and pH-optimum of NSILA-s receptors significantly differed from those of insulin-receptors. A new, highly specific radio-receptor assay was developed, applying 125I NSILA-s and liver cell membranes or lymphocytes. By this means the NSILA-s concentration of blood plasma was determined under normal and pathological (hypoglycaemizing tumours, hypopituritarism, acromegaly, anorexia nervosa, etc.) conditions. It is concluded that, 90% of the NSILA-s concentration of blood plasma is bound. In cases of hypoglycaemizing tumours increased NSILA-s activity was demonstrated both in blood serum and in the extracts of the tumour-tissue. Pharmacological doses of growth hormon (GH) increased plasma NSILA-s concentration, however, in the case of stimulation- and inhibition-tests carried out in normal patients, no unambiguous relationship could be demonstrated between plasma GH- and NSILA-s-levels. (L.E.)

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

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

  4. Growth hormone, insulin-like growth factor system and carcinogenesis.

    Boguszewski, Cesar Luiz; Boguszewski, Margaret Cristina da Silva; Kopchick, John J

    2016-01-01

    The growth hormone (GH) and insulin-like growth factor (IGF) system plays an important role in the regulation of cell proliferation, differentiation, apoptosis, and angiogenesis. In terms of cell cycle regulation, the GH-IGF system induces signalling pathways for cell growth that compete with other signalling systems that result in cell death; thus the final effect of these opposed forces is critical for normal and abnormal cell growth. The association of the GH-IGF system with carcinogenesis has long been hypothesised, mainly based on in vitro studies and the use of a variety of animal models of human cancer, and also on epidemiological and clinical evidence in humans. While ample experimental evidence supports a role of the GH-IGF system in tumour promotion and progression, with several of its components being currently tested as central targets for cancer therapy, the strength of evidence from patients with acromegaly, GH deficiency, or treated with GH is much weaker. In this review, we will attempt to consolidate this data. (Endokrynol Pol 2016; 67 (4): 414-426). PMID:27387246

  5. Stereotactic Irradiation of GH-Secreting Pituitary Adenomas

    G. Minniti

    2012-01-01

    Full Text Available Radiotherapy (RT is often employed in patients with acromegaly refractory to medical and/or surgical interventions in order to prevent tumour regrowth and normalize elevated GH and IGF-I levels. It achieves tumour control and hormone normalization up to 90% and 70% of patients at 10–15 years. Despite the excellent tumour control, conventional RT is associated with a potential risk of developing late toxicity, especially hypopituitarism, and its role in the management of patients with GH-secreting pituitary adenomas remains a matter of debate. Stereotactic techniques have been developed with the aim to deliver more localized irradiation and minimize the long-term consequences of treatment, while improving its efficacy. Stereotactic irradiation can be given in a single dose as stereotactic radiosurgery (SRS or in multiple doses as fractionated stereotactic radiotherapy (FSRT. We have reviewed the recent published literature on stereotactic techniques for GH-secreting pituitary tumors with the aim to define the efficacy and potential adverse effects of each of these techniques.

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

    Elisa Verrua

    2014-01-01

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

  7. A rapid and simple assay for growth hormone-binding protein activity in human plasma

    The newly discovered circulating growth hormone binding proteins dictate a re-evaluation of the state of GH in plasma in health and disease as the binding proteins are known to affect GH metabolism and action. We describe a rapid and simple GH-binding assay that allows determination of free and complexed plasma GH, as well as GH-binding protein activity as an index of GH-binding protein levels, with relative ease. The method is based on incubation of plasma with 125I-GH and separation of bound from free GH on small DEAE-cellulose columns; it can be used on a large scale for routine determinations. The results obtained by this method are comparable to those obtained with the previously used slow and more cumbersome gel filtration technique. Initial data obtained in normal subject and certain disease states show that the bound fraction of plasma GH is similar in men, women and children, is unaffected by pregnancy or acute infection, but is marginally decreased in liver cirrhosis. In acromegaly, binding protein activity also appears normal when allowance is made for partial saturation of the binding proteins by the high prevailing GH levels. The technique we describe should facilitate investigations of normal and abnormal regulation of the GH binding proteins. (author)

  8. Rational Design of Potent Antagonists to the Human Growth Hormone Receptor

    Fuh, Germaine; Cunningham, Brian C.; Fukunaga, Rikiro; Nagata, Shigekazu; Goeddel, David V.; Wells, James A.

    1992-06-01

    A hybrid receptor was constructed that contained the extracellular binding domain of the human growth hormone (hGH) receptor linked to the transmembrane and intracellular domains of the murine granulocyte colony-stimulating factor receptor. Addition of hGH to a myeloid leukemia cell line (FDC-P1) that expressed the hybrid receptor caused proliferation of these cells. The mechanism for signal transduction of the hybrid receptor required dimerization because monoclonal antibodies to the hGH receptor were agonists whereas their monovalent fragments were not. Receptor dimerization occurs sequentially-a receptor binds to site 1 on hGH, and then a second receptor molecule binds to site 2 on hGH. On the basis of this sequential mechanism, which may occur in many other cytokine receptors, inactive hGH analogs were designed that were potent antagonists to hGH-induced cell proliferation. Such antagonists could be useful for treating clinical conditions of hGH excess, such as acromegaly.

  9. Gamma irradiation effects on human growth hormone producing pituitary adenoma tissue

    Irradiation-induced effects on pituitary cell morphology and secretion of growth hormone (GH) and prolactin (PRL) have been analysed using an in vitro system. Specimens for organ culture were were obtained from three patients with pituitary tumours causing acromegaly but with different clinical activity of disease. Specimens were followed in vitro 1 h - 6 days after single-dose gamma irradiation (60Co) with 70 100 and 150 Gy, respectively. These doses are used in clinical work for the stereotactic radiosuregery of pituitary adenomas. Considerable fluctuations in hormone secretion/release occurred during the first 24h after irradiation. All three tumours showed individual differences concern ing irradiation-induced morphological damage. Only a minor variation occurred between specimens from the same tumour. An individual sensitivity to irradiation of pituitary tumours in vitro is documented. The great number of surviving pituitary tumour cells one week after irradiation-many with an intact ultrastructure and containing hormone granules-indicated an initial high degree of radioresistance. (author)

  10. ENDOSCOPIC ENDONASAL TRANS-SPHENOIDAL SURGERY: OUR EXPERIENCE OF 40 PITUITARY ADENOMAS TREATED AT A TERTIARY CENTRE

    Pankaj

    2015-12-01

    Full Text Available Introduction of Endoscope has revolutionized the surgical management of Pituitary tumors. Endoscopic endonasal approach has now become the most acceptable and routinely done procedure. In our tertiary center, we have analysed the data of 40 patients, who underwent endoscopic endonasal surgery over a period of one year. Clinical parameters, tumor excision, endocrinological status, complication rates were retrospectively analysed. One month follow up with repeat MRI and hormonal status was compared with pre-operative findings. There were 15 functioning and 25 non-functioning pituitary adenomas. There were 6 prolactinomas, 4 acromegaly, 4 Cushing and 1 FSH secreting pituitary adenoma. 25 were non-functioning adenomas. There was one microadenoma with Cushing syndrome. Subtotal excision was done in 76% of non-functioning and 66% of functioning adenomas. The remission rates were 80% in non-functional and almost 75% in functional pituitary adenoma. In our series, endoscopic approach proved to be less invasive, excellent tumors removal rate and better post-operative results. The complications were also less and comparable to other conventional methods. But it has steep learning curve and needs technical skills.

  11. Functional Evaluation of TSH Secretory Reserve Capacity in Hypothalamo pituitary Disorders

    The TRH stimulation test was known as a highly diagnostic method in hypothalamo pituitary disorders. To evaluate the location and the extension of the lesion, we estimated TSH response to TRH test in 27 patients. Correlation between volume of sella and TSH response was also studied. The results obtained were 25 follows: 1) In Sheehan's syndrome, TSH response after TRH test were not observed in all of 12 patients. 2) All 2 acromegaly patients showed normal TSH response. 3) In 4 cases of chromophobe adenoma, 2 cases showed no TSH response. In 2 responded cases, one patient whose tumor mass extended to suprasella region was hypothyroid state. 4) In craniopharyingioma 3 cases, the tumor which extended to intrasella showed hypothyroid and no TSH response. 5) Correlation between volume of sella and TSH response were valuable in 2 cases, but no diagnostic significance. 6) In diabetes inspidus, TSH response were all absent. 7) In primary amenorrhea, TSH response observed in 1 case, which conformed with isolated FSH deficiency.

  12. Management of Obstructive Sleep Apnea

    V K Vijayan

    2014-03-01

    Full Text Available Obstructive Sleep Apnea (OSA is an important public health problem and is associatedwith considerable morbidity and mortality. Therefore, treatment of this condition is ofparamount importance. The treatment of OSA includes general and behaviouralmeasures, mechanical measures including continuous positive airway pressure(CPAP, Bilevel positive airway pressure (BiPAP and Oral Appliances (OA,pharmacological treatment and surgical procedures. Continuous positive airwaypressure (CPAP treatment reverses the repetitive upper airway obstruction of sleepapnea and associated daytime sleepiness and is the most effective treatment for OSA.However maintaining patient adherence to CPAP therapy is a challenge. Weight lossshould be recommended to overweight patients with OSA, as it has been shown thatweight reduction has additional health benefits. Treatment of underlying medicalconditions such as hypothyroidism or acromegaly has profound effect onapnea/hypopnea index. A subset of patients with OSA may benefit from supplementaloxygen and positional therapy. Presently, there are no effective pharmacotherapeuticagents for treatment of patients with OSA and the role of surgical treatment in OSA iscontroversial. However, pharmacological treatment of persisting residual sleepiness,despite adequate positive airway pressure therapy delivery and adherence, is indicatedand may improve daytime sleepiness.Key words : CPAP, Oral appliances, Modafinil, CPAP complianceUvulopalatopharyngoplasty, positional therapy

  13. Effect of subcutaneous injection of a long-acting analogue of somatostatin (SMS 201-995) on plasma thyroid-stimulating hormone in normal human subjects

    SMS 201-995 (SMS), a synthetic analogue of somatostatin (SRIF) has been shown to be effective in the treatment of the hypersecretion of hormones such as in acromegaly. However, little is known about the effects of SMS on the secretion of thyroid-stimulating hormone (TSH) in normal subjects. In this study, plasma TSH was determined with a highly sensitive immunoradiometric assay, in addition to the concentration of SMS in plasma and urine with a radioimmunoassay, following subcutaneous injection of 25, 50, 100 μg of SMS or a placebo to normal male subjects, at 0900 h after an overnight fast. The plasma concentrations of SMS were dose-responsive and the peak levels were 1.61 +/- 0.09, 4.91 +/- 0.30 and 8.52 +/- 1.18 ng/ml, which were observed at 30, 15 and 45 min after the injection of 25, 50, and 100 μg of SMS, respectively. Mean plasma disappearance half-time of SMS was estimated to be 110 +/- 3 min. Plasma TSH was suppressed in a dose dependent manner and the suppression lasted for at least 8 hours. At 8 hours after the injection of 25, 50, and 100 μg of SMS, the plasma TSH levels were 43.8 +/- 19.4, 33.9 +/- 9.4 and 24.9 +/- 3.2%, respectively, of the basal values

  14. Investigation of Responsiveness to Thyrotropin-Releasing Hormone in Growth Hormone-Producing Pituitary Adenomas

    Sang Ouk Chin

    2013-01-01

    Full Text Available Objective. The aim of this study was to investigate how the paradoxical response of GH secretion to TRH changes according to tumor volumes. Methods. Patients with newly diagnosed acromegaly were classified as either TRH responders or nonresponders according to the results of a TRH stimulation test (TST, and their clinical characteristics were compared according to responsiveness to TRH and tumor volumes. Results. A total of 41 acromegalic patients who underwent the TST were included in this study. Between TRH responders and nonresponders, basal GH, IGF-I levels, peak GH levels, and tumor volume were not significantly different, but the between-group difference of GH levels remained near significant over the entire TST time. during the TST were significantly different according to the responsiveness to TRH. Peak GH levels and during the TST showed significantly positive correlations with tumor volume with higher levels in macroadenomas than in microadenomas. GH levels over the entire TST time also remained significantly higher in macroadenomas than in microadenomas. Conclusion. Our data demonstrated that the paradoxical response of GH secretion to TRH in GH-producing pituitary adenomas was not inversely correlated with tumor volumes.

  15. Angioid streaks, clinical course, complications, and current therapeutic management

    Ilias Georgalas

    2008-12-01

    Full Text Available Ilias Georgalas1, Dimitris Papaconstantinou2, Chrysanthi Koutsandrea2, George Kalantzis2, Dimitris Karagiannis2, Gerasimos Georgopoulos2, Ioannis Ladas21Department of Ophthalmology, “G. Gennimatas” Hospital of Athens, NHS, Athens, Greece; 2Department of Ophthalmology, “G. Gennimatas” Hospital of Athens, University of Athens, Athens, GreeceAbstract: Angioid streaks are visible irregular crack-like dehiscences in Bruch’s membrane that are associated with atrophic degeneration of the overlying retinal pigmented epithelium. Angioid streaks may be associated with pseudoxanthoma elasticum, Paget’s disease, sickle-cell anemia, acromegaly, Ehlers–Danlos syndrome, and diabetes mellitus, but also appear in patients without any systemic disease. Patients with angioid streaks are generally asymptomatic, unless the lesions extend towards the foveola or develop complications such as traumatic Bruch’s membrane rupture or macular choroidal neovascularization (CNV. The visual prognosis in patients with CNV secondary to angioid streaks if untreated, is poor and most treatment modalities, until recently, have failed to limit the devastating impact of CNV in central vision. However, it is likely that treatment with antivascular endothelial growth factor, especially in treatment-naive eyes to yield favorable results in the future and this has to be investigated in future studies.Keywords: angioid streaks, pseudoxanthoma elasticum, choroidal neovascularization

  16. The CT findings of ganglioglioma

    The authors report 2 cases of gangliogliomas of the sellar region, one of which was associated with chromophobe adenoma. Case 1. A 14-year-old female was admitted for a visual disturbance. Neurological examination showed bitemporal hemianopsia and secondary optic atrophy. CT scan showed an isodense mass in the suprasellar region, with homogneous enhancement by contrast medium and an accompanying low-density area in the left thalamus adjacent to the enhanced tumor mass. The histological diagnosis of the surgical specimen was ganglioglioma. Case 2. A 52-year-old female was admitted under the diagnosis of pituitary adenoma. Neurological examination showed bitemporal hemianopsia and signs of acromegaly. Plain skull X-ray film showed sellar ballooning and decalcification of the posterior clinoid process. The tumor resected in the first operation was histologically diagnosed as chromophobe adenoma mixed with ganglioglioma. In the follow-up, she showed signs and symptoms of a recurrent tumor, which was revealed to be ganglioglioma without any recurrence of chromophobe adenoma in the subsequent operations. The CT scan before the second operation showed an isodense supraand intrasellar mass with marked enhancement. Ganglioglioma is a rare, benign, nerve-cell tumor, mostly occurring in childhood and adolescence. The CT findings, together with the clinical history, the age of the patient, and the tumor location, may suggest the tentative preoperative diagnosis of ganglioglioma. (author)

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

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

    1987-10-01

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

  18. Radioimmunoassay for determination of alpha subunit of pituitary glycoprotein hormones in patients with pituitary tumors

    A radioimmunoassay method for alpha subunit has been described and applied for serum alpha subunit determinations in normal subjects and 71 patients with pituitary tumors /45 acromegalic and 26 non-acromegalic/. The labelling of alpha subunit by the chloramine T technique yielded 125I-alpha subunit of high specific activity and high immuno-reactivity. Three purification methods of labelled 125I-alpha subunit were compared; the best separation of undamaged 125I-alpha subunit from impurities was achieved by gel filtration on Ultrogel AcA54 column, whereas gel filtration on Sephadex G-100 and adsorption chromatography on CF-11 cellulose gave less satisfactory results. Microheterogenity of 125I-alpha subunit was disclosed by chromatofocusing on PBE 94; the fractions of high immunoreactivitiy had isoelectric points of 6.0, 5.5 and 4.8. In normal subjects, radioimmunoassay of alpha subunit gave the following results /mean and SD/: 0.75 ng/ml +- 0.41 in males and 0.80 ng/ml +- 0.39 in females in reproductive age. In 9 acromegalic serum alpha subunit concentration were elevated up to 21 ng/ml, and in 8 non-acromegalic up to 30 ng/ml. One woman with acromegaly and high serum alpha subunit concentration had also elevated serum TSH associated with hyperthyroids. Our results disclosed that high serum alpha subunit concentration occurs in 25 % of patients with pituitary adenomas. (Author)

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

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

  20. The value of radiation therapy for pituitary tumors

    Following points are discussed in this review. 1) Historical review of our previous therapeutic management. 2) Classification of pituitary adenomas. 3) Clinical analysis of my recent 58 cases. 4) Verification of usefulness of postoperative irradiation which achieved to increase in local control rate. 5) Authoritativeness of radiotherapy. In general, 3 to 4 portal technique or arc therapy were employed. The lateral opposing field technique was avoid to use. The recommended doses using linear accelerator x-ray technique is approximately 5000 cGy in 5 weeks. To prevent radiation hazard; (1) examiner should not use technique of two opposed fields, (2) total doses should not exceed 5000 cGy in 5 to 6 weeks and the use of daily fractions should not exceed 200 cGy. 6) Correlation of hormone secreting tumors and radiation therapy. 7) Problem of radiosurgery and heavy particle. 8) Countermeasure for recurrence cases. 9) Problem of side effects of radiotherapy and its precaution. Complication of radiation for pituitary adenoma found that the significant side effects are negligibly small in recent years. 10) Pituitary tumor are originally slow growing and benign tumor, therefore the response to irradiation takes long time to elapse for final evaluation. For instance, over 80 to 90% of acromegaly patients respond HGH successfully, but this may require from one to several years. 11) Conclusion. (author)

  1. Visceral Adiposity Index: An Indicator of Adipose Tissue Dysfunction

    Marco Calogero Amato

    2014-01-01

    Full Text Available The Visceral Adiposity Index (VAI has recently proven to be an indicator of adipose distribution and function that indirectly expresses cardiometabolic risk. In addition, VAI has been proposed as a useful tool for early detection of a condition of cardiometabolic risk before it develops into an overt metabolic syndrome. The application of the VAI in particular populations of patients (women with polycystic ovary syndrome, patients with acromegaly, patients with NAFLD/NASH, patients with HCV hepatitis, patients with type 2 diabetes, and general population has produced interesting results, which have led to the hypothesis that the VAI could be considered a marker of adipose tissue dysfunction. Unfortunately, in some cases, on the same patient population, there is conflicting evidence. We think that this could be mainly due to a lack of knowledge of the application limits of the index, on the part of various authors, and to having applied the VAI in non-Caucasian populations. Future prospective studies could certainly better define the possible usefulness of the VAI as a predictor of cardiometabolic risk.

  2. Botulinum neurotoxin: Progress in negating its neurotoxicity; and in extending its therapeutic utility via molecular engineering. MiniReview.

    Kostrzewa, Richard M; Kostrzewa, Rose Anna; Kostrzewa, John P

    2015-10-01

    While the poisonous effects of botulinum neurotoxin (BoNT) have been recognized since antiquity, the overall actions and mechanisms of effects of BoNT have been elucidated primarily over the past several decades. The general utility of BoNT is described in the paper, but the focus is mainly on the approaches towards negating the toxic effects of BoNT, and on the projection of an engineered BoNT molecule serving as a Trojan Horse to deliver a therapeutic load for treatment of a host of medical disorders. The BoNT molecule is configured with a binding domain, a zinc-dependent protease with specificity primarily for vesicular proteins, and a translocation domain for delivery of the metalloprotease into the cytoplasm. The anti-toxin approaches for BoNT include the use of vaccines, antibodies, block of BoNT binding or translocation, inhibition of metalloprotease activity, impeded translocation of the protease/catalytic domain, and inhibition of the downstream Src signaling pathway. Projections of BoNT as a therapeutic include its targeting to non-cholinergic nerves, also targeting to non-neuronal cells for treatment of hypersecretory disorders (e.g., cystic fibrosis), and treatment of hormonal disorders (e.g., acromegaly). Still in the exploratory phase, there is the expectation of major advances in BoNT neuroprotective strategies and burgeoning utility of engineered BoNTs as therapeutics. PMID:26192475

  3. Carpal Fusion

    Jalal Jalalshokouhi*

    2012-05-01

    Full Text Available Carpal fusion may be seen in hereditary and nonhereditary conditions such as acrocallosal syndrome,acromegaly, Apert syndrome, arthrogryposis, Carpenter syndrome, chromosomal abnormalities, ectrodactyly-ectodermal dysplasia-cleft (EEC syndrome, the F form of acropectorovertebral dysgenesis or the F syndrome, fetal alcohol syndrome, Holt-Oram syndrome, Leopard syndrome, multiple synostosis syndrome, oligosyndactyly syndrome, Pfeiffer-like syndrome, scleroderma, split hand and foot malformation, Stickler syndrome, thalidomide embryopathy, Turner syndrome and many other conditions as mentioned in Rubinstein-Taybi's book. Sometimes there is no known causative disease.Diagnosis is usually made by plain X-ray during studying a syndrome or congenital disease or could be an incidental finding like our patients. Hand bone anomalies are more common in syndromes or other congenital or non-hereditary conditions, but polydactyly, syndactyly or oligodactyly and carpal fusions are interesting. X-ray is the modality of choice, but MRI and X-ray CT with multiplanar reconstructions may be used for diagnosis.

  4. Pegvisomant-Induced Cholestatic Hepatitis in an Acromegalic Patient with UGT1A1 ​⁎ 28 Mutation

    Maria Susana Mallea-Gil

    2016-01-01

    Full Text Available Pegvisomant (PEGv is a growth hormone receptor antagonist approved for the treatment of acromegaly; one of its documented adverse effects is reversible elevation of hepatic enzymes. We report a 39-year-old male acromegalic patient with a pituitary macroadenoma who underwent transsphenoidal surgery. The patient’s condition improved but GH and IGF-I levels did not normalize; as a consequence, we first administered dopamine agonists and then somatostatin receptor ligands (SRLs with poor response. PEGv 15 mg every other day was added to lanreotide 120 mg monthly. The patient developed a severe hepatitis five months after starting the combination therapy. Elevated ferritin, iron, and transferrin saturation suggested probable hepatitis due to haemochromatosis. We performed a liver biopsy which showed an acute cholestatic hepatitis consistent with toxic etiology. A heterozygous genotype UGT1A1​⁎28 polymorphism associated with Gilbert’s syndrome was also found in this Argentine patient. The predominant clinical presentation resembled an acute cholestatic hepatitis associated with severe hemosiderosis, a different and new pattern of PEGv hepatotoxicity.

  5. Aglepristone: A review on its clinical use in animals.

    Gogny, Anne; Fiéni, Francis

    2016-03-01

    Aglepristone (RU 46534) is a competitive progesterone antagonist that is indicated for the treatment of various progesterone-dependent physiological or pathologic conditions. Aglepristone has proven to be an effective means of terminating pregnancy in most species. When used to induce parturition, aglepristone was effective in all cases in the bitch, cow, and goat, with no apparent adverse effects on neonatal health or milk production. When used to schedule an elective cesarean section, aglepristone treatment was deemed safe for dams and puppies, providing that the ovulation date had been accurately assessed at the time of breeding. Irrespective of the stage of pregnancy at injection, treatment with aglepristone has no apparent negative effects on subsequent fertility. Aglepristone is also a safe and relatively effective means of treating pyometra. However, given the high level of septic risk and the likelihood of rapid deterioration, such therapy is not recommended in emergency situations. Treatment of feline fibroadenomatosis using aglepristone has given promising results, but repeat treatment may be necessary in cats previously treated with long-acting progestagens. The use of aglepristone in other progesterone-dependent diseases has yet to be fully evaluated but may prove valuable, especially in the treatment of insulin-resistant diabetes mellitus, acromegaly, and the treatment of some vaginal tumors in the bitch. PMID:26525399

  6. The sesamoid index in psoriatic arthropathy

    Whitehouse, Richard W.; Aslam, Rizwan [Manchester Royal Infirmary, Department of Clinical Radiology, Manchester (United Kingdom); Bukhari, Marwan [Manchester Royal Infirmary, Department of Rheumatology, Manchester (United Kingdom); Groves, Clare; Cassar-Pullicino, Victor [Agnes Hunt and Robert Jones Hospital, Department of Radiology, Oswestry (United Kingdom)

    2005-04-01

    The sesamoid index was originally described as an aid to the diagnosis of acromegaly. We performed this study to assess the value of the thumb sesamoid index in the diagnosis of psoriatic arthropathy. Retrospective measurement of the sesamoid index (length x width of the medial thumb sesamoid), along with the age and sex were recorded for patients as described below. Patients with psoriasis were subdivided into those with or without radiographic evidence of hand arthropathy. Fifty-nine consecutive patients attending rheumatology clinics with arthralgia and psoriasis were studied. Comparison groups with radiographic evidence of rheumatoid arthritis (52 patients), osteoarthritis (44) or normal hands (55) were also recorded. Twenty-one of 59 patients with psoriasis and arthropathy had a sesamoid index >40, compared with two of 52 with rheumatoid arthritis, none of 44 with osteoarthritis and none of 55 normals. Psoriatic arthropathy is a recognised cause of bone enlargement, usually in the phalanges due to periostitis and proliferative enthesopathy. We have confirmed that psoriatic hand arthropathy can cause significant enlargement of the thumb sesamoids, a feature which is easily quantified and may assist diagnosis. (orig.)

  7. The sesamoid index in psoriatic arthropathy

    The sesamoid index was originally described as an aid to the diagnosis of acromegaly. We performed this study to assess the value of the thumb sesamoid index in the diagnosis of psoriatic arthropathy. Retrospective measurement of the sesamoid index (length x width of the medial thumb sesamoid), along with the age and sex were recorded for patients as described below. Patients with psoriasis were subdivided into those with or without radiographic evidence of hand arthropathy. Fifty-nine consecutive patients attending rheumatology clinics with arthralgia and psoriasis were studied. Comparison groups with radiographic evidence of rheumatoid arthritis (52 patients), osteoarthritis (44) or normal hands (55) were also recorded. Twenty-one of 59 patients with psoriasis and arthropathy had a sesamoid index >40, compared with two of 52 with rheumatoid arthritis, none of 44 with osteoarthritis and none of 55 normals. Psoriatic arthropathy is a recognised cause of bone enlargement, usually in the phalanges due to periostitis and proliferative enthesopathy. We have confirmed that psoriatic hand arthropathy can cause significant enlargement of the thumb sesamoids, a feature which is easily quantified and may assist diagnosis. (orig.)

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

    Chen-Ku Chih Hao

    2004-10-01

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

  9. Enfoque terapéutico en 154 pacientes con acromegalia Therapeutic management in 154 acromegalic patients

    Marcos P. Manavela

    2010-08-01

    Full Text Available La acromegalia es una enfermedad poco frecuente producida en más del 95% de los casos por un tumor hipofisario secretor de hormona de crecimiento (GH. Las manifestaciones clínicas están asociadas a síntomas locales por crecimiento del tumor o a las consecuencias orgánicas y metabólicas secundarias a la hipersecreción de GH. Debido a la alta morbilidad y mortalidad asociadas a la acromegalia, un tratamiento individualizado y optimizado para cada paciente es fundamental. Informamos el enfoque terapéutico de nuestro servicio de endocrinología en la atención de 154 pacientes con acromegalia. Utilizando criterios bioquímicos estrictos, con la cirugía logramos un 32% de remisión global, tasa relativamente baja debido fundamentalmente a que la mayor parte de los pacientes presentaban macroadenomas con un alto porcentaje de invasividad local. Con radioterapia complementaria o como tratamiento inicial se logró la remisión en el 65.4% de los pacientes irradiados. El 14.0% de los pacientes controlaron la enfermedad utilizando agonistas dopaminérgicos solos o combinados con otra droga, mientras que aquellos que utilizaron análogos de la somatostatina normalizaron los parámetros bioquímicos en un 45.7% de los casos. En conclusión, con los diferentes tratamientos utilizados obtuvimos el control de la acromegalia en el 55.2% de los casos, esperando optimizar el tratamiento de estos pacientes en la medida en que contemos con y tengamos acceso a nuevas herramientas terapéuticas.Acromegaly is a chronic, invalidating disease due in over 95% of cases to a growth hormone (GH secreting pituitary adenoma. Its clinical manifestations are associated to local complications related to the tumor growth and/or to the metabolic consequences of GH excess. We report here our experience on 154 acromegalic patients. Surgical remission rate using stringent biochemical criteria was 32%, a figure relatively low due to the great number of patients bearing

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

    MARCELO MIRANDA

    1998-09-01

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

  11. Contemporary issues in the evaluation and management of pituitary adenomas.

    Pekic, S; Stojanovic, M; Popovic, V

    2015-12-01

    Pituitary adenomas are common benign monoclonal neoplasms accounting for about 15% of intracranial neoplasms. Data from postmortem studies and imaging studies suggest that 1 of 5 individuals in the general population may have pituitary adenoma. Some pituitary adenomas (mainly microadenomas which have a diameter of less than 1 cm) are exceedingly common and are incidentally diagnosed on magnetic resonance imaging (MRI) performed for an unrelated reason (headache, vertigo, head trauma). Most microadenomas remain clinically occult and stable in size, without an increase in tumor cells and without local mass effects. However, some pituitary adenomas grow slowly, enlarge by expansion and become demarcated from normal pituitary (macroadenomas have a diameter greater than 1 cm). They may be clinically silent or secrete anterior pituitary hormones in excess such as prolactin, growth hormone (GH), or adrenocorticotropic hormone (ACTH) causing diseases like prolactinoma, acromegaly, Cushing's disease or rarely thyroid-stimulating hormone (TSH) or gonadotropins (LH, FSH). The incidence of the various subtypes of pituitary adenoma varies but the most common is prolactinoma. Clinically non-functioning pituitary adenomas (NFPAs), which do not secrete hormones often cause local mass symptoms and represent one-third of pituitary adenomas. Given the high prevalence of pituitary adenomas and their heterogeneity (different tumor subtypes), it is critical that clinicians have a thorough understanding of the potential abnormalities in pituitary function and prognostic factors for behavior of pituitary adenomas in order to timely implement specific treatment modalities. Regarding pathogenesis of these tumors genetics, epigenetics and signaling pathways are the focus of current research yet our understanding of pituitary tumorigenesis remains incomplete. Although several genes and signaling pathways have been identified as important factors in the development of pituitary tumors, current

  12. Considering GH replacement for GH-deficient adults with a previous history of cancer: a conundrum for the clinician.

    Yuen, Kevin C J; Heaney, Anthony P; Popovic, Vera

    2016-05-01

    Previous studies have shown that GH and IGF-I may enhance tumorigenesis, metastasis, and cell proliferation in humans and animals. Evidence supporting this notion is derived from animal model studies, epidemiological studies, experience from patients with acromegaly, molecular therapeutic manipulation of GH and IGF-I actions, and individuals with GH receptor and congenital IGF-I deficiencies. Prior exposure to radiation therapy, aging, family history of cancer, and individual susceptibility may also contribute to increase this risk. Therefore, the use of GH replacement in patients with a history of cancer raises hypothetical safety concerns for patients, caregivers, and providers. Studies of GH therapy in GH-deficient adults with hypopituitarism and childhood cancer survivors have not convincingly demonstrated an increased cancer risk. Conversely, the risk of occurrence of a second neoplasm (SN) in childhood cancer survivors may be increased, with meningiomas being the most common tumor; however, this risk appears to decline over time. In light of these findings, if GH replacement is to be considered in patients with a previous history of cancer, we propose this consideration to be based on each individual circumstance and that such therapy should only be initiated at least 2 years after cancer remission is achieved with the understanding that in some patients (particularly those with childhood cancers), GH may potentially increase the risk of SNs. In addition, close surveillance should be undertaken working closely with the patient's oncologist. More long-term data are thus needed to determine if GH replacement in GH-deficient adults with a history of cancer is associated with the development of de novo tumors and tumor recurrence. PMID:26732039

  13. No differences in metabolic outcomes between nadir GH 0.4 and 1.0 ng/mL during OGTT in surgically cured acromegalic patients (observational study).

    Ku, Cheol Ryong; Choe, Eun Yeong; Hong, Jae Won; Kim, Eui Hyun; Park, Se Hee; Kim, Sun Ho; Lee, Eun Jig

    2016-06-01

    Metabolic impairment is the common cause for mortality in acromegalic patients. In this study, long-term improvements of metabolic parameters were evaluated according to 2 different remission criteria.This was an observational cohort study before and up to 1 year after transsphenoidal adenomectomy (TSA). Participants were 187 patients with acromegaly. At 6 months after TSA, remitted patients with age- and sex-matched normalized IGF-1 were divided into 2 groups: remission 1 (R1), nadir growth hormone (GH) below 0.4 ng/mL; and remission 2 (R2), nadir GH between 0.4 and 1.0 ng/mL in oral glucose tolerance test (OGTT). Metabolic parameters during serial OGTTs were evaluated for 12 months. Remission was achieved in 157 (R1-136; R2-21) patients. Immediate postoperative metabolic parameters including body weight, body mass index, glucose, insulin, and free fatty acid in OGTT were all significantly improved in R1 and R2. HOMA-%β and HOMA-IR scores also improved in both R1 and R2. These improvements persisted for duration (12 months) of this study. However, no difference was present in metabolic parameters between R1 and R2. Although the patients with preoperative adrenal insufficiency presented significantly increased HOMA scores before TSA, there was no difference between classifications of deficient pituitary axes and changes of metabolic parameters after TSA. Remitted patients exhibited rapid restoration of metabolic parameters immediate postoperative period. Long-term improvements in metabolic parameters were not different between the 2 different nadir GH cut-offs, 0.4 and 1.0 ng/mL. PMID:27310957

  14. Medical application of in vivo neutron activation analysis

    Cohn, S.H.; Ellis, K.J.; Vartsky, D.; Zanzi, I.; Aloia, J.F.

    1978-01-01

    The clinical usefulness of total body neutron activation analysis (TBNAA) was clearly established at an IAEA panel meeting in Vienna in 1972. It is best demonstrated by the studies involving the measurement of total-body calcium. This measurement provides data useful for the diagnosis and management of metabolic bone disorders. It should be emphasized, however, that while most of the applications to date have involved calcium and phosphorus, the measurement of sodium, chlorine and nitrogen also appear to be useful clinically. Total-body calcium measurements utilizing TBNAA have been used in studies of osteoporosis to establish absolute and relative deficits of calcium in patients with this disease in comparison to a normal contrast population. Changes in total-body calcium (skeletal mass) have also been useful for quantitating the efficacy of various therapies in osteoporosis. Serial measurements over periods of years provide long-term balance data by direct measurement with a higher precision (+- 2%) than is possible by the use of any other technique. In the renal osteodystrophy observed in patients with renal failure, disorders of both calcium and phosphorus, as well as electrolyte disturbances, have been studied. The measure of total-body levels of these elements gives the clinician useful data upon which to design dialysis therapy. The measurement of bone changes in endocrine dysfunction has been studied, particularly in patients with thyroid and parathyroid disorders. In parathyroidectomy, the measurement of total-body calcium, post-operatively, can indicate the degree of bone resorption. Skeletal metabolism and body composition in acromegaly and Cushing's disease have also been investigated by TBNAA. Levels of cadmium in liver and kidney have also been measured in-vivo by prompt-gamma neutron activation and associated with hypertension, emphysema and cigarette smoking.

  15. A possible new syndrome with growth-hormone secreting pituitary adenoma, colonic polyposis, lipomatosis, lentigines and renal carcinoma in association with familial testicular germ cell malignancy: A case report

    Mai Phuong L

    2007-03-01

    Full Text Available Abstract Background Germ-cell testicular cancer has not been definitively linked to any known hereditary cancer susceptibility disorder. Familial testicular cancer in the presence of other findings in affected and unaffected family members might indicate a previously-unidentified hereditary cancer syndrome. Case presentation The patient was diagnosed with a left testicular seminoma at age 28, and treated with left orchiectomy followed by adjuvant cobalt radiation. His family history is significant for testicular seminoma in his son, bladder cancer in his sister, and lipomatosis in his father. His evaluation as part of an etiologic study of familial testicular cancer revealed multiple colon polyps (adenomatous, hyperplastic, and hamartomatous first found in his 50 s, multiple lipomas, multiple hyperpigmented skin lesions, left kidney cancer diagnosed at age 64, and a growth-hormone producing pituitary adenoma with associated acromegaly diagnosed at age 64. The patient underwent genetic testing for Cowden syndrome (PTEN gene, Carney complex (PRKAR1A gene, and multiple endocrine neoplasia syndrome type 1 (MEN1 gene; no deleterious mutations were identified. Discussion The constellation of benign and malignant neoplasms in the context of this patient's familial testicular cancer raised the possibility that these might be manifestations of a known hereditary susceptibility cancer syndrome; however, genetic testing for the three syndromes that were most likely to explain these findings did not show any mutation. Alternatively, this family's phenotype might represent a novel neoplasm susceptibility disorder. This possibility cannot be evaluated definitively on the basis of a single case report; additional observations and studies are necessary to investigate this hypothesis further.

  16. Indium-111 pentetreotide single-photon emission tomography in patients with TSH-secreting pituitary adenomas: correlation with the effect of a single administration of octreotide on serum TSH levels

    Few data are available on the visualization of somatostatin receptors in vivo in patients with thyrotropin (TSH)-secreting adenoma. We studied five patients with TSH-secreting adenomas using single-photon emission tomography (SPET) after administration of indium-111 pentetreotide. The intensity of 111In-pentetreotide uptake by the tumours was correlated with the degree of TSH suppression after a single administration of 100 μg octreotide s.c. Five patients (three women and two men) aged 27-46 years were investigated. Except for one patient with acromegaly, all had pure TSH-secreting tumours. One patient was previously untreated, while two had received octreotide, one antithyroid drugs, and one radioiodine. In all patients SPET demonstrated increased uptake of 111In-pentetreotide by the pituitary adenoma. The target to non-target ratio (T/nT) of 111In-pentetreotide uptake was higher than 10 in three patients. Administration of 100 μg octreotide s.c. caused a significant reduction in TSH levels from 4.8±1.4 mU/l to a nadir of 3.1±1.1 mU/l after 6 h (P111In-pentetreotide scan visualized somatostatin receptors in all five of the patients with TSH-secreting pituitary adenomas, confirming the frequent presence of somatostatin receptors in these rare tumours, even though the correlation with the TSH inhibition after a single administration of octreotide did not reach significance. (orig.). With 1 fig., 1 tab

  17. The physiology of growth hormone and sport.

    Widdowson, W Matthew

    2012-02-01

    The growth hormone (GH)\\/ insulin-like growth factor-I (IGF-I) axis exerts short-and long-term metabolic effects that are potentially important during exercise. Exercise is a potent stimulus to GH release and there is some evidence that the acute increase in GH is important in regulating substrate metabolism post-exercise. Regular exercise also increases 24-hour GH secretion rates, which potentially contributes to the physiologic changes induced by training. The effects of GH replacement in GH-deficient adults provide a useful model with which to study the effects of the more long-term effects of the GH\\/ IGF-I axis. There is convincing evidence that GH replacement increases exercise capacity. Measures of exercise performance including maximal oxygen uptake (VO2max) and ventilatory threshold (VeT) are impaired in GH deficiency and improved by GH replacement, probably through some combination of increased oxygen delivery to exercising muscle, increased fatty acid availability with glycogen sparing, increased muscle strength, improved body composition and improved thermoregulation. Administration of supraphysiologic doses of GH to athletes increases fatty acid availability and reduces oxidative protein loss particularly during exercise, and increases lean body mass. It is not known whether these effects translate to improved athletic performance, although recombinant human GH is known to be widely abused in sport. The model of acromegaly provides evidence that long-term GH excess does not result in improved performance but it is possible that a "window" exists in which the protein anabolic effects of supraphysiologic GH might be advantageous.

  18. Traditional and novel aspects of the metabolic actions of growth hormone.

    Sperling, Mark A

    2016-06-01

    Growth hormone has been known to be diabetogenic for almost a century and it's diabetogenic properties fostered consideration of excessive and abnormal GH secretion as a cause of diabetes, as well as a role in the microvascular complications, especially retinopathy. However, besides inducing insulin resistance, GH also is lipolytic and a major anabolic hormone for nitrogen retention and protein synthesis. These actions are best illustrated at the extremes of GH secretion: Gigantism/acromegaly is characterized by excessive growth, CHO intolerance, hyperplasia of bone, little body fat and prominent muscle development, whereas total deficiency of GH secretion or action is associated with adiposity, poor growth, and poor muscle development. These actions also become apparent during puberty and pregnancy, times when GH secretion is increased and account for the characteristic changes in body composition and tendency to diabetes. More recently, tissue specific deletions of the GH receptor (GHR), have uncovered newer metabolic effects including it's essential role in triglyceride export from the liver when GHR is deleted in the liver, leading to hepatic steatosis and ultimately to hepatic adenoma formation, effects which may explain these findings in obesity, a state of diminished GH secretion and action. In addition deletion of GH action in muscle and fat is associated with specific patterns of disturbed phenotype and metabolic effects in CHO, fat, and protein metabolism affecting the specific tissue and whole body function. This chapter provides an overview of these classic and newer metabolic functions of GH, placing this hormone and its actions in a central role of body fuel economy in health and disease. PMID:26194064

  19. Biological and medical research with accelerated heavy ions at the Bevalac, 1974--1977. [Planning for use for radiotherapy and as radiation source for diagnostic radiography

    Elam, S. (ed.)

    1977-04-01

    The Bevalac, a versatile high-energy heavy-ion accelerator complex, has been in operation for less than two years. A major purpose for which the Bevalac was constructed was to explore the possibility of heavy-ion teams for therapy for certain forms of cancer. Significant progress has been made in this direction. The National Cancer Institute has recognized the advantages that these and other accelerated particles offer, and heavy ions have been included in a long-term plan for particle therapy that will assess by means of controlled therapeutic tests the value of various modalities. Since accelerated heavy ions became available, the possibility of other contributions, not planned, became apparent. We are developig a new diagnostic method known as heavy-ion radiography that has greatly increased sensitivity for soft-tissue detail and that may become a powerful tool for localizing early tumors and metastases. We have discovered that radioactive beams are formed from fragmentation of stable deflected beams. Use of these autoradioactive beams is just beginning; however, we know that these beams will be helpful in localizing the region in the body where therapy is being delivered. In addition, it has been demonstrated that instant implantation of the radioactive beam allows direct measurements of blood perfusion rates in inaccessible parts of the body, and such a technique may become a new tool for the study of fast hot atom reactions in biochemistry, tracer biology and nuclear medicine. The Bevalac will also be useful for the continuation of previously developed methods for the control of acromegaly, Cushing's disease and, on a research basis, advanced diabetes mellitus with vascular disease. The ability to make small bloodless lesions in the brain and elsewhere with heavy-ion beams has great potential for nervous-system studies and perhaps later for radioneurosurgery.

  20. Medical application of in-vivo neutron activation analysis at Brookhaven National Laboratory

    Total-body calcium measurements utilizing TBNAA have been used in studies of osteoporosis to establish absolute and relative deficits of calcium in patients with this disease in comparison to a normal contrast population. Changes in total-body calcium (skeletal mass) have also been useful for quantitating the efficacy of various therapies in osteoporosis. Serial measurements over periods of years provide long-term balance data by direct measurement with a higher precision (+-2%) than is possible by the use of any other technique. In the renal osteodystrophy observed in patients with renal failure, disorders of both calcium and phosphorus, as well as electrolyte disturbances, have been studied. The measurement of bone changes in endocrine dysfunction have been studied, particularly in patients with thyroid and parathyroid disorders. In parathyroidectomy, the measurement of total-body calcium, post-operatively, can indicate the degree of bone resorption. Skeletal metabolism and body composition in acromegaly and Cushing's disease have also been investigated by TBNAA. Levels of cadmium in liver and kidney have also been measured in vivo by prompt-gamma neutron activation and associated with hypertension, emphysema and cigarette smoking. Total-body nitrogen and potassium measurements serve as indices of muscle mass and are useful in studies of the interrelation of cancer, diet and nutrition. An essential requirement in these studies is the in-vivo measurement of changes in body composition, primarily revealed by nitrogen content. Currently the optimal method for measurement of total-body nitrogen is prompt-gamma neutron activation. There can be little question that in-vivo neutron activation is a useful addition to the techniques for medical research which provides new and previously unavailable information

  1. Clinical profile and response to treatment of patients with pituitary adenomas submitted to radiotherapy

    Objective: to evaluate the clinical profile of patients with pituitary adenoma and their response to radiotherapy. Material and method: retrospective study with 22 patients with diagnosis of pituitary adenoma which were submitted to radiotherapy between March 2004 and December 2008. Patients' characteristics such as gender, age, clinical presentation, surgical approach, immunohistochemistry profile, dose of radiation and the response to therapy were analyzed using hormonal dosages and imaging exams. Results: the median age was 51 years and equally distributed in both genders. The tumors were divided according to the Hardy's classification: 27.5% had grade II, 27.5% had grade III and 45% had grade IV. The main symptoms presented by patients at diagnosis were visual impairment in 77% of cases, headache in 68%, amenorrhea and acromegaly in 27% and galactorrhoea in 4.5%. Transphenoidal surgery was performed in 21 patients and only 1 patient was submitted to transcranial approach; 91% of cases had partial resection. Concerning to immunohistochemistry, the expression of ACTH was the most frequent, being present in 41% of cases. The patients were treated in megavoltage equipment mostly with 6 MV linear accelerator. The total radiation dose was 45 Gy in 68% of patients and a dose of 50.4 Gy in 13% of cases. Three-dimensional planning was used in 20 patients. The median follow-up was 41 months. Laboratory and imaging improvement were observed in 73% of patients, stability in 22.5%, and worsening in 4.5%. Conclusion: the results show good rates of response and control of pituitary adenomas by radiation in the first four years after treatment. Considering it has a slow response to treatment, there is a high chance of improvement in results later during the follow-up. (author)

  2. Carney complex (CNC

    Bertherat Jérôme

    2006-06-01

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

  3. Clinical profile and response to treatment of patients with pituitary adenomas submitted to radiotherapy; Avaliacao do perfil e resposta ao tratamento de pacientes portadores de adenoma hipofisario submetidos a radioterapia

    Moraes, Paulo Lazaro de; Freire, Geison Moreira; Dias, Rodrigo Souza; Segreto, Roberto Araujo; Segreto, Helena Regina Comodo [Universidade Federal de Sao Paulo (UNIFESP), Sao Paulo, SP (Brazil). Escola Paulista de Medicina. Setor de Radioterapia], e-mail: segreto.dmed@epm.br; Giordani, Adelmo Jose [Pontificia Univ. Catolica de Sao Paulo, SP (Brazil); Abucham Filho, Julio Zaki [Universidade Federal de Sao Paulo (UNIFESP), Sao Paulo, SP (Brazil). Dept. de Endocrinologia

    2008-12-15

    Objective: to evaluate the clinical profile of patients with pituitary adenoma and their response to radiotherapy. Material and method: retrospective study with 22 patients with diagnosis of pituitary adenoma which were submitted to radiotherapy between March 2004 and December 2008. Patients' characteristics such as gender, age, clinical presentation, surgical approach, immunohistochemistry profile, dose of radiation and the response to therapy were analyzed using hormonal dosages and imaging exams. Results: the median age was 51 years and equally distributed in both genders. The tumors were divided according to the Hardy's classification: 27.5% had grade II, 27.5% had grade III and 45% had grade IV. The main symptoms presented by patients at diagnosis were visual impairment in 77% of cases, headache in 68%, amenorrhea and acromegaly in 27% and galactorrhoea in 4.5%. Transphenoidal surgery was performed in 21 patients and only 1 patient was submitted to transcranial approach; 91% of cases had partial resection. Concerning to immunohistochemistry, the expression of ACTH was the most frequent, being present in 41% of cases. The patients were treated in megavoltage equipment mostly with 6 MV linear accelerator. The total radiation dose was 45 Gy in 68% of patients and a dose of 50.4 Gy in 13% of cases. Three-dimensional planning was used in 20 patients. The median follow-up was 41 months. Laboratory and imaging improvement were observed in 73% of patients, stability in 22.5%, and worsening in 4.5%. Conclusion: the results show good rates of response and control of pituitary adenomas by radiation in the first four years after treatment. Considering it has a slow response to treatment, there is a high chance of improvement in results later during the follow-up. (author)

  4. Predicting difficult laryngoscopy in acromegalic patients undergoing surgery for excision of pituitary tumors: A comparison of extended Mallampati score with modified Mallampati classification

    Ashish Bindra

    2013-01-01

    Full Text Available Background: There are numerous reports of difficult laryngoscopy and intubation in patients with acromegaly. To date, no study has assessed the application of extended Mallampati score (EMS for predicting difficult intubation in acromegalics. The primary aim of this study was to compare EMS with modified Mallampati classification (MMP in predicting difficult laryngoscopy in acromegalic patients. We hypothesized that since EMS has been reported to be more specific and better predictor than MMP, it may be superior to the MMP to predict difficult laryngoscopy in acromegalic patients. Materials and Methods: For this prospective cohort study with matched controls, acromegalic patients scheduled to undergo pituitary surgery over a period of 3 years (January 2008-December 2010 were enrolled. Preoperative airway assessment was performed by experienced anesthesiologists and involved a MMP and the EMS. Under anesthesia, laryngoscopic view was assessed using Cormack-Lehane (CL grading. MMP and CL grades of I and II were defined "easy" and III and IV as "difficult". EMS grade of I and II were defined "easy" and III as "difficult". Data were used to determine the sensitivity, specificity, positive predictive value, negative predictive value, and accuracy of MMP and EMS in predicting difficult laryngoscopy. Results: Seventy eight patients participated in the study (39 patients in each group. Both MMP and EMS failed to detect difficult laryngoscopy in seven patients. Only one laryngoscopy was predicted to be difficult by both tests which was in fact, difficult. Conclusion: We found that addition of neck extension did not improve the predictive value of MMP.

  5. Somatic mosaicism underlies X-linked acrogigantism syndrome in sporadic male subjects.

    Daly, Adrian F; Yuan, Bo; Fina, Frederic; Caberg, Jean-Hubert; Trivellin, Giampaolo; Rostomyan, Liliya; de Herder, Wouter W; Naves, Luciana A; Metzger, Daniel; Cuny, Thomas; Rabl, Wolfgang; Shah, Nalini; Jaffrain-Rea, Marie-Lise; Zatelli, Maria Chiara; Faucz, Fabio R; Castermans, Emilie; Nanni-Metellus, Isabelle; Lodish, Maya; Muhammad, Ammar; Palmeira, Leonor; Potorac, Iulia; Mantovani, Giovanna; Neggers, Sebastian J; Klein, Marc; Barlier, Anne; Liu, Pengfei; Ouafik, L'Houcine; Bours, Vincent; Lupski, James R; Stratakis, Constantine A; Beckers, Albert

    2016-04-01

    Somatic mosaicism has been implicated as a causative mechanism in a number of genetic and genomic disorders. X-linked acrogigantism (XLAG) syndrome is a recently characterized genomic form of pediatric gigantism due to aggressive pituitary tumors that is caused by submicroscopic chromosome Xq26.3 duplications that includeGPR101 We studied XLAG syndrome patients (n= 18) to determine if somatic mosaicism contributed to the genomic pathophysiology. Eighteen subjects with XLAG syndrome caused by Xq26.3 duplications were identified using high-definition array comparative genomic hybridization (HD-aCGH). We noted that males with XLAG had a decreased log2ratio (LR) compared with expected values, suggesting potential mosaicism, whereas females showed no such decrease. Compared with familial male XLAG cases, sporadic males had more marked evidence for mosaicism, with levels of Xq26.3 duplication between 16.1 and 53.8%. These characteristics were replicated using a novel, personalized breakpoint junction-specific quantification droplet digital polymerase chain reaction (ddPCR) technique. Using a separate ddPCR technique, we studied the feasibility of identifying XLAG syndrome cases in a distinct patient population of 64 unrelated subjects with acromegaly/gigantism, and identified one female gigantism patient who had had increased copy number variation (CNV) threshold forGPR101that was subsequently diagnosed as having XLAG syndrome on HD-aCGH. Employing a combination of HD-aCGH and novel ddPCR approaches, we have demonstrated, for the first time, that XLAG syndrome can be caused by variable degrees of somatic mosaicism for duplications at chromosome Xq26.3. Somatic mosaicism was shown to occur in sporadic males but not in females with XLAG syndrome, although the clinical characteristics of the disease were similarly severe in both sexes. PMID:26935837

  6. [Treatment of pituitary adenomas].

    Mezosi, Emese; Nemes, Orsolya

    2009-09-27

    According to epidemiological studies, the prevalence of pituitary adenomas is 16.5% and the majority of them are "incidentalomas". The symptoms of pituitary disorders are often non-specific; disturbances of pituitary function, compression symptoms, hypophysis apoplexy or accidental findings may help the diagnosis. The hormonal evaluation of pituitary adenomas is different from the algorithm used in the disorders of peripheral endocrine organs. The first-line therapy of prolactinomas are the dopamine agonists, and the aims of the treatment are to normalize the prolactin level, restore fertility in child-bearing age, decrease tumor mass, save or improve the residual pituitary function and inhibit the relapse of the disease. The available dopamine agonists in Hungary are bromocriptine and quinagolide. In case of tumors with good therapeutic response, medical therapy can be withdrawn after 3-5 years; hyperprolactinemia will not recur in 2/3 of these patients. Neurosurgery is the primary therapy of GH-, ACTH-, TSH-producing and inactive adenomas. In the last decades, significant improvement has been reached in surgical procedures, resulting in low mortality rates. Acromegalic patients with unresectable tumors have a great benefit from somatostatin analog treatment. The growth hormone receptor antagonist pegvisomant is the newest modality for the treatment of acromegaly. The medical therapy of Cushing's disease is still based on the inhibition of steroid production. A new, promising somatostatin analog, pasireotide is evaluated in clinical trials. The rare TSH-producing tumor can respond to both dopamine agonist and somatostatin analog therapy. The application of conventional radiotherapy has decreased; radiotherapy is mainly used in the treatment of invasive, incurable or malignant tumors. Further studies are needed to elucidate the exact role of radiosurgery and fractionated stereotaxic irradiation in the treatment of pituitary tumors. PMID:19758960

  7. MRI of growth hormone-secreting pituitary adenomas: factors determining pretreatment hormone levels

    Preoperative serum growth hormone (GH) level is one of the most important determinants of outcome. Our aim was to assess MRI findings which may correlate with pretreatment GH levels in GH-secreting adenomas. We retrospectively studied 29 patients with acromegaly caused by a pituitary adenoma. Tumor size (height, width, thickness and volume), suprasellar extension, sphenoid or cavernous sinus invasion, signal intensity and contrast enhancement were studied. Linear regression analysis or Fisher's exact probability test was used for statistical analysis. Factors related to high GH levels were the maximum dimension of the tumour (r = 0.496, P < 0.01), its volume (r = 0.439, P < 0.05), spenoid sinus invasion (P < 0.01) and intracavernous carotid artery encasement (P < 0.01). The other items were not related to serum GH levels. Since we believe surgery is the first choice of treatment and the cavernous sinus is difficult of access with a conventional surgical approach, preoperative assessment of invasion into the cavernous sinus is critical for predicting the surgical outcome. Low GH levels (5-50 ng/ml) were found with tumours medial to the intercarotid line and high levels (more than 101 ng/ml) with invasive tumours with carotid artery encasement. Variable GH levels were noted with tumours extending beyond the intercarotid line. Because functioning adenomas invading the cavernous sinus tend to have markedly high hormone levels, and only patients with carotid artery encasement showed markedly elevated GH levels, we believe carotid artery encasement a reliable MRI indicator of cavernous sinus invasion. (orig.)

  8. Heterogeneity of pituitary and plasma prolactin in man: decreased affinity of big prolactin in a radioreceptor assay and evidence for its secretion

    Molecular heterogeneity of immunoreactive human PRL (IR-hPRL) plasma was assessed by exclusion chromatography in blood from 4 normal adults, 3 newborn infants, 2 late gestational women, 3 patients with primary hypothyroidism and high PRL levels, 2 with functional hyperprolactinemia, 3 with acromegaly, and 10 with PRL-secreting tumors. Three forms of PRL were detected: big-big hPRL, big hPRL, and little hPRL. In normal subjects, the proportion of big-big, big, and little hPRL components was 5.1%, 9.1%, and 85.8%, respectively, without change in the distribution after TRF stimulation. In 8 of 10 patients with PRL-secreting tumors, we detected a significantly higher proportion of big PRL. In 2 additional patients with prolactinomas, the proportion of big PRL was much higher. In 3 of 10 patients, the molecular heterogeneity of the tumor PRL was similar to that in plasma. In 1 acromegalic, there was a very high proportion of big-big hPRL. The PRL fractions were tested in a radioreceptor assay (RRA) using membranes from rabbit mammary gland. Big PRL was much less active than little PRL in the RRA. The fractions were rechromatographed after storage. Big PRL partially distributed as little or big-big PRL, while little PRL remained unchanged. Big-big PRL from tumor extract partially converted into big and little PRL. The big PRL obtained by rechromatography had low activity in the RRA. These observations suggest at least part of the receptor activity of big PRL may arise from generation of or contamination by little PRL. The decreased binding affinity of big PRL in the RRA also indicates that big PRL has little, if any, biological activity. The evidence suggests big PRL is a native PRL dimer linked by intermolecular disulfide bonds which arises in the lactotrope as a postsynthetic product or derivative and is not a true precursor prohormone

  9. Heterogeneity of pituitary and plasma prolactin in man: decreased affinity of big prolactin in a radioreceptor assay and evidence for its secretion

    Garnier, P.E.; Aubert, M.L.; Kaplan, S.L.; Grumbach, M.M.

    1978-12-01

    Molecular heterogeneity of immunoreactive human PRL (IR-hPRL) plasma was assessed by exclusion chromatography in blood from 4 normal adults, 3 newborn infants, 2 late gestational women, 3 patients with primary hypothyroidism and high PRL levels, 2 with functional hyperprolactinemia, 3 with acromegaly, and 10 with PRL-secreting tumors. Three forms of PRL were detected: big-big hPRL, big hPRL, and little hPRL. In normal subjects, the proportion of big-big, big, and little hPRL components was 5.1%, 9.1%, and 85.8%, respectively, without change in the distribution after TRF stimulation. In 8 of 10 patients with PRL-secreting tumors, we detected a significantly higher proportion of big PRL. In 2 additional patients with prolactinomas, the proportion of big PRL was much higher. In 3 of 10 patients, the molecular heterogeneity of the tumor PRL was similar to that in plasma. In 1 acromegalic, there was a very high proportion of big-big hPRL. The PRL fractions were tested in a radioreceptor assay (RRA) using membranes from rabbit mammary gland. Big PRL was much less active than little PRL in the RRA. The fractions were rechromatographed after storage. Big PRL partially distributed as little or big-big PRL, while little PRL remained unchanged. Big-big PRL from tumor extract partially converted into big and little PRL. The big PRL obtained by rechromatography had low activity in the RRA. These observations suggest at least part of the receptor activity of big PRL may arise from generation of or contamination by little PRL. The decreased binding affinity of big PRL in the RRA also indicates that big PRL has little, if any, biological activity. The evidence suggests big PRL is a native PRL dimer linked by intermolecular disulfide bonds which arises in the lactotrope as a postsynthetic product or derivative and is not a true precursor prohormone.

  10. Medical application of in vivo neutron activation analysis

    The clinical usefulness of total body neutron activation analysis (TBNAA) was clearly established at an IAEA panel meeting in Vienna in 1972. It is best demonstrated by the studies involving the measurement of total-body calcium. This measurement provides data useful for the diagnosis and management of metabolic bone disorders. It should be emphasized, however, that while most of the applications to date have involved calcium and phosphorus, the measurement of sodium, chlorine and nitrogen also appear to be useful clinically. Total-body calcium measurements utilizing TBNAA have been used in studies of osteoporosis to establish absolute and relative deficits of calcium in patients with this disease in comparison to a normal contrast population. Changes in total-body calcium (skeletal mass) have also been useful for quantitating the efficacy of various therapies in osteoporosis. Serial measurements over periods of years provide long-term balance data by direct measurement with a higher precision (+- 2%) than is possible by the use of any other technique. In the renal osteodystrophy observed in patients with renal failure, disorders of both calcium and phosphorus, as well as electrolyte disturbances, have been studied. The measure of total-body levels of these elements gives the clinician useful data upon which to design dialysis therapy. The measurement of bone changes in endocrine dysfunction has been studied, particularly in patients with thyroid and parathyroid disorders. In parathyroidectomy, the measurement of total-body calcium, post-operatively, can indicate the degree of bone resorption. Skeletal metabolism and body composition in acromegaly and Cushing's disease have also been investigated by TBNAA. Levels of cadmium in liver and kidney have also been measured in-vivo by prompt-gamma neutron activation and associated with hypertension, emphysema and cigarette smoking

  11. Critical review of the clinical relevance of growth hormone and its measurement in the nuclear medicine laboratory

    A wide variety of metabolic and stressful stimuli, both physical and psychologic, produce rapid elevation of plasma growth hormone (GH). In addition, spontaneous elevation of GH occurs during the day, and a rise in GH occurs in association with the initial slow-wave sleep episode at night. Although the identity of the GH releasing factor has not yet been established, a hypothalamic factor inhibiting GH release named somatostatin identified and synthesized. Most, if not all, of the GH rises are mediated by neural mechanisms, and therefore they may be disrupted by many disease processes affecting the pituitary or the hypothalamus. In acromegaly, hypersecretion of GH occurs, and remnants of hypothalamic control can frequently be demonstrated, suggesting a hypothalamic origin for at least some cases. Provocative stimuli commonly used to assess adequacy of GH responses include hypoglycemia, arginine infusion, and exercise. Administration of L-dopa or apomorphine also produces GH elevation, and since these agents may activate specific dopamine mechanisms, they are of particular interest. Two comprehensive commercial kits have been evaluated, and three major defects have been identified. The literature provided in both kits was inadequate. Both kits required an initial dilution without any replication, so that dilution error would be undetected. One of the kits did not provide a full complement of materials. Quantities of HGH antigen and antisera sufficient for large numbers of assays are also available commercially. However, no commercial source was found for control sera containing known quantities of HGH. Individual laboratories to provide their own supply of sera to use for quality control. It is now known that GH exists in multiple forms in plasma and that antisera may differ in ability to bind these forms. It will, therefore, be necessary for laboratories to revalidate the assay if a different antiserum is used. (U.S.)

  12. Evaluation of long-acting somatostatin analog injection devices by nurses: a quantitative study

    Adelman DT

    2012-12-01

    Full Text Available Daphne T Adelman,1 Andrea Burgess,2 Philippa R Davies,31Feinberg School of Medicine, Northwestern University, Chicago, IL, USA; 2Neuroendocrine Tumor Department, The Christie, Manchester, UK; 3Neuroendocrine Tumor Unit, Royal Free Hospital, London, UKAbstract: The somatostatin analogs (SSAs lanreotide Autogel/Depot and octreotide long-acting release are used to treat acromegaly and neuroendocrine tumors. The present study evaluated opinions on SSA injection devices, including a recently approved lanreotide new device (lanreotide-ND, among nurses in Europe and the USA. Nurses injecting SSAs for at least three patients per year (n = 77 were interviewed regarding SSA devices. Device attributes were rated via questionnaire; nurses were then timed administering test injections with lanreotide-ND and octreotide long-acting release. The most important delivery system attributes were easy/convenient preparation and injection (ranked in the top five by 70% of nurses, low clogging risk (58%, and high product efficacy (55%. Compared with the octreotide long-acting release device, lanreotide-ND scored higher on 15/16 attributes, had shorter mean preparation and administration time (329 versus 66 seconds, respectively; P ≤ 0.01 and a higher overall preference score (70 versus 114, respectively; P ≤ 0.01. The five most important lanreotide-ND attributes were: prefilled device, confidence a full dose was delivered, low clogging risk, easy/convenient preparation and injection, and fast administration. These device features could lead to improvements in clinical practice and benefit patients/caregivers who administer SSAs at home.Keywords: nurse, somatostatin analog, device, lanreotide, octreotide

  13. The Technique and Dosimetry of Pituitary Implantation Using Sources of Y90

    Pituitary ablation by needle implantation of Y90 is finding increasing use in the treatment of breast and prostatic cancer, as well as diabetic retinopathy, Cushing's disease, acromegaly, and perhaps exophthalmos in Graves' disease. Yttrium-90 is the most suitable radioisotope when complete ablation of the gland is sought. This is because only β-particles are emitted, the maximum range (7mm) of which is comparable with the dimensions of the gland. The implantation of rods of standard activity into the gland, irrespective of its size, does not permit a standard dose level to be delivered to the gland and the method of implantation is to select the size and activity of the source to fit the dimensions of the gland in question. Thus consistency in procedure may be attempted from one implant to another. The shape of the gland and the mode of access to it is such that complete destruction may conveniently be obtained by implanting two.sources. Each source is a rod of sintered Y2O3, (2-mm diam., and of length cut to suit the individual gland length). The rod activity is also selected to suit the gland dimensions: typically, it is from 2 to 3 me. Radiation dose has been experimentally related to geometry and activity. Mix D wax is used as the tissue- equivalent absorber, film as the detector and a calibrated Sr90 source (which decays into Y90) as the standard. One outcome of this work is that the pituitary gland requires a radiation dose of between 100 000 and 200 000 rad for necrosis and ablation. (author)

  14. Biological and medical research with accelerated heavy ions at the Bevalac, 1974--1977

    The Bevalac, a versatile high-energy heavy-ion accelerator complex, has been in operation for less than two years. A major purpose for which the Bevalac was constructed was to explore the possibility of heavy-ion teams for therapy for certain forms of cancer. Significant progress has been made in this direction. The National Cancer Institute has recognized the advantages that these and other accelerated particles offer, and heavy ions have been included in a long-term plan for particle therapy that will assess by means of controlled therapeutic tests the value of various modalities. Since accelerated heavy ions became available, the possibility of other contributions, not planned, became apparent. We are developig a new diagnostic method known as heavy-ion radiography that has greatly increased sensitivity for soft-tissue detail and that may become a powerful tool for localizing early tumors and metastases. We have discovered that radioactive beams are formed from fragmentation of stable deflected beams. Use of these autoradioactive beams is just beginning; however, we know that these beams will be helpful in localizing the region in the body where therapy is being delivered. In addition, it has been demonstrated that instant implantation of the radioactive beam allows direct measurements of blood perfusion rates in inaccessible parts of the body, and such a technique may become a new tool for the study of fast hot atom reactions in biochemistry, tracer biology and nuclear medicine. The Bevalac will also be useful for the continuation of previously developed methods for the control of acromegaly, Cushing's disease and, on a research basis, advanced diabetes mellitus with vascular disease. The ability to make small bloodless lesions in the brain and elsewhere with heavy-ion beams has great potential for nervous-system studies and perhaps later for radioneurosurgery

  15. Spectrum of disorders leading to hyperprolactinaemia

    Objective: To determine the frequency of disorders leading to hyperprolactinaemia (HP) in patients who reported to AFIP Rawalpindi. Study Design: Cross- sectional study. Place and duration of study: Department of Chemical Pathology and Endocrinology, Armed Forces Institute of Pathology Rawalpindi, from January to June 2011. Patients and Methods: Patients with serum prolactin levels > 530 ml U/l in females and 360 ml U/l in males were included. Patients with hyperprolactinaemia(Hp) due to physiological causes (pregnancy and lactation etc), drug induced, irradiation and hypothyroid patients on thyroxin treatment were excluded. Seventy six samples were collected from the patients for the workup of pathological conditions. Serum prolactin, FSH, LH, estradiol, testosterone, GH, cortisol, TSH and free T4 were analysed on Immulite 2000, while LFTs and RFTs on Hitachi. Pituitary adenomas were confirmed by MRI. Results: Seventy six patients had HP due to pathological causes, 13(17%) males and 63(83%) females had mean age of 30+-11 years. Pituitary microadenoma was the cause of hyperprolactinemia in 30 (39.5%) cases, pituitary macroadenoma in 12 (15.8%), subclinical hypothyroidism in 14 (18.4%), primary hypothyroidism in 10 (13.2%), PCOS in 4 (5.3%), cirrhosis in 2 (2.6%), idiopathic in 2 (2.6%), CKD in 1 (1.3%) and acromegaly in 1 (1.3%) patient. HP was significantly correlated with size of prolactinoma and serum TSH levels (primary and subclinical hypothyroidism) (p value < 0.05). Conclusion: It is concluded that prolactinoma is the commonest pathology causing hyperprolactinemia, followed by hypothyroidism and PCOS in patients who reported to AFIP Rawalpindi. This will help in early diagnosis along with further management of the patient. (author)

  16. In vivo detection of somatostatin receptors in patients with functionless pituitary adenomas by means of a radioiodinated analog of somatostatin ([123I]SDZ 204-090)

    The recent availability of a Tyr3-substituted octreotide (SDZ 204-090) for radioiodination has allowed somatostatin (SRIH) receptor binding to be studied in vivo, and receptor-positive tumors of different origins to be visualized with a gamma-camera. This prompted us to investigate whether this compound could be used for external imaging of functionless pituitary adenomas displaying SRIH receptors. Eight patients with functionless pituitary adenomas, three patients with acromegaly, and three with macroprolactinoma were injected iv with 123I-labeled Tyr3-octreotide and then scanned with a gamma-camera. Positive scans were obtained in the three acromegalics and in two of the eight patients with functionless pituitary tumors. The patients with macroprolactinoma had negative scans. The diagnosis of functionless pituitary adenomas was confirmed by light and electron microscopic examination as well as immunocytochemical studies. In vitro binding of [125I]Tyr11-SRIH to cell membranes was evaluated in four functionless and three GH-secreting adenomas removed from seven of the patients. All of the GH-secreting as well as one of the four functionless adenomas had high affinity SRIH-binding sites, without differences in number or affinity, whereas SRIH-binding sites were not detected in the others. Positive scans were observed only in patients bearing tumors with high affinity SRIH-binding sites. In conclusion, [123I]Tyr3-octreotide appears to be a promising tool for singling out, in vivo, patients with functionless pituitary tumors displaying SRIH receptors who might potentially benefit from octreotide treatment

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

    Jackson Gondim

    2003-09-01

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

  18. Comparative evaluation of several parameters of glycemic homeostasis in patients with functional pituitary adenomas

    Miroslava Rostislavovna Mikityuk

    2014-03-01

    Full Text Available Aim. To investigate glycemic homeostasis in patients with acromegaly and hyperprolactinaemia due to functional pituitary adenoma.Materials and Methods. We enrolled 48 patients with somatotropinoma (Group 1, 19 patients with somatomammotropinoma (Group 2 and 24 patients with prolactinoma (Group 3 with no history of glycemic disorders. Median age was 48.63±9.98 years; median disease duration was 13.1±6.9 years. The comparison group was composed of overweight or obese patients (n=75. We conducted a comparative analysis of glycemic parameters (Glc0, immunoreactive insulin, HOMA2_S%, HOMA2_B% и HOMA2_IR, Ssum and S under isolated segments of the glycemic curve. We also tested their correlation with plasma levels of growth hormone, prolactin and IGF-1.Results. We detected hyperinsulinemia (>20 μU/mL in 35.1% of participants from Group 1, 33.3% and 25% from groups 2 and 3, respectively. There was no statistically significant difference between glycemic parameters of the study groups, though patients were mildly insulin resistant (HOMA2_IR 1.88 to 2.38 units independantly from tumour secretory activity. Groups 1 and 2 were characterized by significantly higher fasting blood glucose levels vs. comparison group (though still within the reference values, and glucose homeostasis was maintained with lower activity of pancreatic β-cells. Average Ssumanalysis showed lower glucose tolerance in groups 1 and 2 vs. Group 3. Median S values under isolated glycemic curve segments were significantly higher than in comparison group. Plasma levels of growth hormone did not correlate with Ssum and S parameters in groups 1 and 2. There was a linear correlation between Ssum and plasma IGF-1 levels in patients from Group 1 (r=0.45; p=0.006. We also noted that Ssum had a non-linear correlation with plasma prolactin levels in patients from Group 3 (r=0.41; p<0.05.Conclusion. Postprandial area under the glycemic curve has positive linear association with

  19. Molecular screening of pituitary adenomas for gene mutations and rearrangements

    Herman, V.; Drazin, N.Z.; Gonskey, R.; Melmed, S. (Cedars-Sinai Medical Center, Los Angeles, CA (United States))

    1993-07-01

    Although pituitary tumors arise as benign monoclonal neoplasms, genetic alterations have not readily been identified in these adenomas. The authors studied restriction fragment abnormalities involving the GH gene locus, and mutations in the p53 and H-, K-, and N-ras genes in 22 human GH cell adenomas. Twenty two nonsecretory adenomas were also examined for p53 and ras gene mutations. Seven prolactinoma DNA samples were tested for deletions in the multiple endocrine neoplasia-1 (MEN-1) locus, as well as for rearrangements in the hst gene, a member of the fibroblast growth factor family. In DNA from GH-cell adenomas, identical GH restriction patterns were detected in both pituitary and lymphocyte DNA in all patients and in one patient with a mixed GH-TSH cell adenoma. Using polymerase chain reaction (PCR)-single stranded conformation polymorphism analysis, no mutations were detected in exons 5, 6, 7 and 8 of the p53 gene in GH cell adenomas nor in 22 nonsecretory adenomas. Codons 12/13 and 61 of H-ras, K-ras, and N-ras genes were also intact on GH cell adenomas and in nonsecretory adenomas. Site-specific probes for chromosome 11q13 including, PYGM, D11S146, and INT2 were used in 7 sporadic PRL-secreting adenomas to detect deletions of the MEN-1 locus on chromosome 11. One patient was identified with a loss of 11p, and the remaining 6 patients did not demonstrate loss of heterozygosity in the pituitary 11q13 locus, compared to lymphocyte DNA. None of these patients demonstrated hst gene rearrangements which also maps to this locus. These results show that p53 and ras gene mutations are not common events in the pathogenesis of acromegaly and nonsecretory tumors. Although hst gene rearrangements and deletions of 11q13 are not associated with sporadic PRl-cell adenoma formation, a single patient was detected with a partial loss of chromosome 11, including the putative MEN-1 site. 31 refs., 5 figs., 2 tabs.

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

    ALICE HELENA DUTRA VIOLANTE

    1999-09-01

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

  1. Outcomes of Proton Therapy for Patients With Functional Pituitary Adenomas

    Purpose/Objective(s): This study evaluated the efficacy and toxicity of proton therapy for functional pituitary adenomas (FPAs). Methods and Materials: We analyzed 165 patients with FPAs who were treated at a single institution with proton therapy between 1992 and 2012 and had at least 6 months of follow-up. All but 3 patients underwent prior resection, and 14 received prior photon irradiation. Proton stereotactic radiosurgery was used for 92% of patients, with a median dose of 20 Gy(RBE). The remainder received fractionated stereotactic proton therapy. Time to biochemical complete response (CR, defined as ≥3 months of normal laboratory values with no medical treatment), local control, and adverse effects are reported. Results: With a median follow-up time of 4.3 years (range, 0.5-20.6 years) for 144 evaluable patients, the actuarial 3-year CR rate and the median time to CR were 54% and 32 months among 74 patients with Cushing disease (CD), 63% and 27 months among 8 patients with Nelson syndrome (NS), 26% and 62 months among 50 patients with acromegaly, and 22% and 60 months among 9 patients with prolactinomas, respectively. One of 3 patients with thyroid stimulating hormone—secreting tumors achieved CR. Actuarial time to CR was significantly shorter for corticotroph FPAs (CD/NS) compared with other subtypes (P=.001). At a median imaging follow-up time of 43 months, tumor control was 98% among 140 patients. The actuarial 3-year and 5-year rates of development of new hypopituitarism were 45% and 62%, and the median time to deficiency was 40 months. Larger radiosurgery target volume as a continuous variable was a significant predictor of hypopituitarism (adjusted hazard ratio 1.3, P=.004). Four patients had new-onset postradiosurgery seizures suspected to be related to generously defined target volumes. There were no radiation-induced tumors. Conclusions: Proton irradiation is an effective treatment for FPAs, and hypopituitarism remains the primary

  2. Helicobacter pylori infection studies using 14C UBT method

    Carbon 14 (14C) is a naturally occurring radioactive isotope of carbon. The half-life of 14C is about 5,730 years, and it decays by emitting a low energy beta (â) particle of mean energy 49 KeV. The biological half life of 14C is 10 to 12 days. 14C is present in the environment and in all organic materials and behaves in the environment in the same manner as other carbon isotopes. In medicine, 14C can be injected to study abnormalities of metabolism that underlie diabetes, gout, anaemia and acromegaly (adult 'gigantism'), and for diagnosis of Helicobacterpylori (H pylori), The discovery H Pylori by Warren and Marshall in 1982 changed the approach to treat peptic ulcer disease (PUD), Since then H pylori has been the focus of clinical research and debate. The causal relationship between H pylori infection and chronic gastritis is well established, H pylori infection is one of the most common human infections worldwide. This organism has been shown to infect over half of the world's population. By the application of radiation much progress has been made worldwide in the field of medicine. This article presents the application of 14C Urea Breath Test (14C UBT) for the diagnosis of the H pylori bacteria which present in the stomach and duodenum, 14C UBT relies on the urease activity of H pylori to detect the presence of active infection. Board of Radiation and Isotope Technology (BRIT), Mumbai has developed 14C UBT method and already in regular practice in many hospitals across the country. Orally administered 14C urea will be hydrolyzed into ammonia (NH4) and 14C labelled carbon dioxide (14CO2). The presence of a significant amount of (14CO2) in the exhaled breath indicates active H pylori infection. 14C UBT is relatively inexpensive, is easy to perform, and does not require endoscopy. 14C UBT has proved to be one of the most accurate methods for assessing H pylori status. The ionizing radiation dose involved in this test is extremely low, much lower than the

  3. Studies on the Radioimmunoassay of Human Growth Hormone - 2. The plasma HGH concentrations in the various febrile diseases

    The plasma HGH concentrations were assayed in total 138 cases by the radioimmunoassay. The groups of control, typhoid fever, epidemic hemorrhagic fever, tuberculous meningitis and other febrile diseases were studied, also were the groups of hyperthyroidism, acromegaly and hypopituitarism. Insulin stimulation test was performed in control, typhoid fever and hypopituitarism. In the control group, the plasma HGH concentration in fasting (early morning) was 2.06±1.183 mμg/ml and its upper limit was 4.5 mμg/ml. No sexual difference was observed. By the insulin stimulation, plasma HGH concentration had rised to the peak level of 24.1±15.71 mμg/ml, 60 min. after the intravenous insulin injection, then decreased to the normal level progressively. In typhoid fever, fasting HGH concentrations in febrile state and in defeverence were 2.5±1.35 mμg/ml and 2.2±3.32 mμg/ml respectively, showing no significant difference with the control group. However, the levels of individual cases ranged widely, compared with the control group. The response to the insulin stimulation test was similar to the control group. In epidemic hemorrhagic fever. the HGH concentrations in oliguric phase, in diuretic phase and in convalescence were 4.2±3.71 mμg/ml, 2.2±l.30 mμg/ml and 3.4±3.01 mμg/ml respectively. No significant differences were observe when compared to the control, but they showed wide range of plasma HGH levels. In tuberculous meningitis, the fasting HGH concentration was 2.9±51.42 mμg/ml. In the other febrile diseases, the value was 2.5±2.23 mμg/ml. In 4 cases of hypopituitarism, the fasting HGH concentration was 2.3±0.42 mμg/ml and ranged normally. However, the response to the insulin stimulation test was not observed. Very high plasma HGH concentrations were observed in acromegalic patients.

  4. Solid phase radioimmunoassay for plasma testosterone using a plastic microtiter tray

    In order to simplify radioimmunoassay for plasma testosterone and to measure many samples at the same time, a method of solid phase radioimmunoassay utilizing a plastic disposable microtiter tray (DMT) by which chromatography can be omitted was investigated. Other steroids except for 5α-dihydrotestosterone (5α-DHT) had a low degree of cross reactivity with the antiserum. Five α-DHT which could be measured together with testosterone in this assay was not a problem clinically because of its strong androgenic activity. The best standard curve was obtained when the antiserum was diluted to 1:1000. The sensitivity of this assay was 10 pg-tube. The maximal adsorption of antibody to plastic DMT was observed when the pH of the antiserum was within the range of 6.5-9.5 and the precoating time was 24 hr at room temperature. The best pH of the incubation buffer was 0.8, and the antigen-antibody reaction became a plateau when the incubation exceeded 6 hrs. The water blank in this assay was 4.6 +- 2.1 pg/tube. The recovery of testosterone (50, 100, 200 pg) when added to 0.1 ml female plasma was 99 +- 6.8%. Coefficients of variation within assay and between assays were below 11.2% and 20.0%, respectively. Correlation between this method and the dextran-coated charcoal method was fairly good (r=0.938). Plasma testosterone levels in 10 normal males and 12 normal females were 616 +- 202 (mean +- SD) ng/dl and 66 +- 29 (mean +- SD) ng/dl, respectively. The levels were low in patients with hypopituitarism, hypogonadism and acromegaly. They were normal in patients with Cushing's syndrome due to adrenal hyperplasia and adenoma, but they were high in a patient with adrenal carcinoma. In a patient with testicular feminization, the level was 632 ng/dl. This increased after the administration of HCG, and decreased to 127.5 ng/dl after castration. (auth.)

  5. Outcomes of Proton Therapy for Patients With Functional Pituitary Adenomas

    Wattson, Daniel A.; Tanguturi, Shyam K. [Harvard Radiation Oncology Program, Boston, Massachusetts (United States); Spiegel, Daphna Y. [Tufts University School of Medicine, Boston, Massachusetts (United States); Niemierko, Andrzej [Department of Radiation Oncology, Massachusetts General Hospital, Boston, Massachusetts (United States); Biller, Beverly M.K.; Nachtigall, Lisa B. [Neuroendocrine Unit, Massachusetts General Hospital, Boston, Massachusetts (United States); Bussière, Marc R. [Department of Radiation Oncology, Massachusetts General Hospital, Boston, Massachusetts (United States); Swearingen, Brooke; Chapman, Paul H. [Department of Neurosurgery, Massachusetts General Hospital, Boston, Massachusetts (United States); Loeffler, Jay S. [Department of Radiation Oncology, Massachusetts General Hospital, Boston, Massachusetts (United States); Shih, Helen A., E-mail: hshih@partners.org [Department of Radiation Oncology, Massachusetts General Hospital, Boston, Massachusetts (United States)

    2014-11-01

    Purpose/Objective(s): This study evaluated the efficacy and toxicity of proton therapy for functional pituitary adenomas (FPAs). Methods and Materials: We analyzed 165 patients with FPAs who were treated at a single institution with proton therapy between 1992 and 2012 and had at least 6 months of follow-up. All but 3 patients underwent prior resection, and 14 received prior photon irradiation. Proton stereotactic radiosurgery was used for 92% of patients, with a median dose of 20 Gy(RBE). The remainder received fractionated stereotactic proton therapy. Time to biochemical complete response (CR, defined as ≥3 months of normal laboratory values with no medical treatment), local control, and adverse effects are reported. Results: With a median follow-up time of 4.3 years (range, 0.5-20.6 years) for 144 evaluable patients, the actuarial 3-year CR rate and the median time to CR were 54% and 32 months among 74 patients with Cushing disease (CD), 63% and 27 months among 8 patients with Nelson syndrome (NS), 26% and 62 months among 50 patients with acromegaly, and 22% and 60 months among 9 patients with prolactinomas, respectively. One of 3 patients with thyroid stimulating hormone—secreting tumors achieved CR. Actuarial time to CR was significantly shorter for corticotroph FPAs (CD/NS) compared with other subtypes (P=.001). At a median imaging follow-up time of 43 months, tumor control was 98% among 140 patients. The actuarial 3-year and 5-year rates of development of new hypopituitarism were 45% and 62%, and the median time to deficiency was 40 months. Larger radiosurgery target volume as a continuous variable was a significant predictor of hypopituitarism (adjusted hazard ratio 1.3, P=.004). Four patients had new-onset postradiosurgery seizures suspected to be related to generously defined target volumes. There were no radiation-induced tumors. Conclusions: Proton irradiation is an effective treatment for FPAs, and hypopituitarism remains the primary

  6. Radioimmunoassay of human insulin-like growth factor binding protein-3 in serum and its clinical application

    Objective: To establish a specific radioimmunoassay (RIA) for serum level of human insulin-like growth factor binding protein-3 (IGFBP-3) and study serum IGFBP-3 levels in normal adults and some pathophysiological states. Methods: IGFBP-3 antibody was raised and iodinated IGFBP-3 was made with lactoperoxidase as a tracer, then set up the RIA for human serum IGFBP-3. Serum levels of normal adults, patients suffered from acromegaly, growth hormone deficiency (GHD), liver cirrhosis and chronic renal failure (CRF) were tested. Results: The minimal detectable range of this IGFBP-3 RIA was (16.9 +- 2.4) μg/L. The affinity constant is 7.9 x 1010 L/mol, without cross-reactivity with IGFBP-1, IGFBP-2, insulin-like growth factor-1 (IGF-1) and human growth hormone (hGH). The mean rate of recovery was 98.7%, and the mean coefficients of variation for intra- and inter batch-assay were (7.8 +- 3.5)% and (10.8 +- 3.1)% respectively. The mean serum levels of IGFBP-3 in 88 normal adults were (3.1+- 0.5) mg/L (range 2.0-4.0 mg/L), while that in 49 active acromegalics were (16.8 +- 9.9) mg/L (range 5.6-42.8 mg/L) and in 19 CRF were (13.2 +- 3.6) mg/L (range 9.8-25.0 mg/L), none of them was overlapping the normal adult levels. The mean serum IGFBP-3 levels of 129 GHD and 18 liver cirrhosis were (1.6 +-0.9) mg/L (range 0.4-3.7 mg/L) and (2.4+-0.7) mg/L (range 1.2-3.7 mg/L) respectively, all of them were overlapping the lower level of serum IGFBP-3 of normal adults. Conclusions: This RIA is easy to use, can detect wide range of serum IGFBP-3, has high specificity and good reproducibility. Serum IGFBP-3 assay is very useful in diagnosis of the hyper-, normal and hypo-secretory state of hGH

  7. Growth hormone-secreting pituitary adenoma:clinical and MR imaging findings

    acromegaly, headache, visual impairment, and symptoms of hyperprolactinemia, for example. On T1-weighted MR images, all tumor were macroadenoma, and there were no characteristic findings different from those of other pituitary tumors. The volume of a tumor did not correlate with serum hormone level. Immunohistochemical study showed positive growth hormone-secreting cells in a limited number of cases

  8. Growth hormone-secreting pituitary adenoma:clinical and MR imaging findings

    Park, Hong Suk; Chang, Kee Hyun; Han, Moon Hee; Sim, Jung Suk; Lee, Sang Hyun; Song, Jae Uoo; Yoo, In Kyu; Jung, Hee Won; Yeon, Kyung Mo [Seoul National Univ. College of Medicine, Seoul (Korea, Republic of)

    1996-10-01

    acromegaly, headache, visual impairment, and symptoms of hyperprolactinemia, for example. On T1-weighted MR images, all tumor were macroadenoma, and there were no characteristic findings different from those of other pituitary tumors. The volume of a tumor did not correlate with serum hormone level. Immunohistochemical study showed positive growth hormone-secreting cells in a limited number of cases.

  9. Studies on the Radioimmunoassay of Human Growth Hormone - 2. The plasma HGH concentrations in the various febrile diseases

    Lee, Young Woo; Lee, Hong Kyu; Koh, Chang Soon; Lee, Mun Ho [Seoul National University College of Medicine, Seoul (Korea, Republic of)

    1972-03-15

    The plasma HGH concentrations were assayed in total 138 cases by the radioimmunoassay. The groups of control, typhoid fever, epidemic hemorrhagic fever, tuberculous meningitis and other febrile diseases were studied, also were the groups of hyperthyroidism, acromegaly and hypopituitarism. Insulin stimulation test was performed in control, typhoid fever and hypopituitarism. In the control group, the plasma HGH concentration in fasting (early morning) was 2.06+-1.183 m{mu}g/ml and its upper limit was 4.5 m{mu}g/ml. No sexual difference was observed. By the insulin stimulation, plasma HGH concentration had rised to the peak level of 24.1+-15.71 m{mu}g/ml, 60 min. after the intravenous insulin injection, then decreased to the normal level progressively. In typhoid fever, fasting HGH concentrations in febrile state and in defeverence were 2.5+-1.35 m{mu}g/ml and 2.2+-3.32 m{mu}g/ml respectively, showing no significant difference with the control group. However, the levels of individual cases ranged widely, compared with the control group. The response to the insulin stimulation test was similar to the control group. In epidemic hemorrhagic fever. the HGH concentrations in oliguric phase, in diuretic phase and in convalescence were 4.2+-3.71 m{mu}g/ml, 2.2+-l.30 m{mu}g/ml and 3.4+-3.01 m{mu}g/ml respectively. No significant differences were observe when compared to the control, but they showed wide range of plasma HGH levels. In tuberculous meningitis, the fasting HGH concentration was 2.9+-51.42 m{mu}g/ml. In the other febrile diseases, the value was 2.5+-2.23 m{mu}g/ml. In 4 cases of hypopituitarism, the fasting HGH concentration was 2.3+-0.42 m{mu}g/ml and ranged normally. However, the response to the insulin stimulation test was not observed. Very high plasma HGH concentrations were observed in acromegalic patients.

  10. Análisis de un registro de adenomas pituitarios Analysis of a pituitary adenoma registry

    Carolina Albiero

    2010-10-01

    Full Text Available Dada la complejidad que reviste el enfoque diagnóstico y terapéutico de los tumores pituitarios, el registro y análisis de la experiencia clínica acumulada es de gran ayuda en la toma de decisiones. En este trabajo se informan datos clínico-terapéuticos, extraídos de un registro computarizado, sobre 519 de un total de 670 pacientes con adenomas pituitarios. Trescientos cuarenta y cinco fueron mujeres (66% y 174 varones (34%, de 14 a 80 años de edad. El diagnóstico final fue: acromegalia en 176, enfermedad de Cushing en 153, prolactinoma en 101 y adenoma clínicamente no-funcionante (ANF en 89. La edad media al momento del diagnóstico de acromegalia fue 43.9 ± 13.5 (16-80, para enfermedad de Cushing 35.7 ± 12.9 (14-72, para prolactinomas 30.0 ± 13.4 (15-79 y para ANF 52.1 ± 15.2 (17-79 años. La creación de un registro institucional de tumores de hipófisis es un instrumento de gran utilidad para el análisis de la experiencia adquirida y constituye una herramienta valiosa para mejorar la estrategia terapéutica, optimizar la relación costo/beneficio y mejorar el cuidado del paciente. Contribuye a la docencia médica, tanto en el pre como en el posgrado y da base a la realización de trabajos de investigación clínica, aportando a la difusión y transferencia de conocimientos.Collection and analysis of data obtained during the clinical treatment of pituitary tumours are of great utility in the decision making process, when facing clinical situations. We report here data on 519 from 670 patients with pituitary adenomas obtained from a computerized registry. Three hundred and forty five were females (66% and 174 males (34%, aged 14-80. Final diagnosis was acromegaly in 176, Cushing's disease in 153, prolactinoma in 101 and clinically non-functioning adenoma in 89. Mean age at diagnosis was 43.9 ± 13.5 (16-80 for acromegalics, 35.7 ± 12.9 (14-72 for Cushing's, 30.0 ± 13.4 (15-79 for prolactinoma and 52.1 ± 15.2 (17-79, for non

  11. Radiation therapy in the multimodal treatment approach of pituitary adenoma

    In this paper, literature will be reviewed to assess the role of modern radiotherapy and radiosurgery in the management of pituitary adenomas. Material and Methods: Nowadays, magnetic resonance imaging for the definition of the target volume and a real three-dimensional (3-D) treatment planning with field conformation and the possibility for non-coplanar irradiation has to be recommended. Most groups irradiate these benign tumors with single doses of 1.8-2.0 Gy up to a total dose of 45 Gy or 50.4 Gy in extensive parasellar adenomas. Adenomas are mostly small, well circumscribed lesions, and have, therefore, attracted the use of stereotactically guided high-precision irradiation techniques which allow extreme focussing and provide steep dose gradients with selective treatment of the target and optimal protection of the surrounding brain tissue. Results: Radiation therapy controls tumor growth in 80-98% of patients with non-secreting adenomas and 67-89% for endocrine active tumors. Reviewing the recent literature including endocrine active and non-secreting adenomas, irradiated postoperatively or in case of recurrence the 5-, 10- and 15-year local control rates amount 92%, 89% and 79%. In cases of microprolactinoma primary therapy consists of dopamine agonists. Irradiation should be preferred in patients with macroprolactinomas, when drug therapy and/or surgery failed or for patients medically unsuitable for surgery. Reduction and control of prolactin secretion can be achieved in 44-70% of patients. After radiotherapy in acromegaly patients somatomedin-C and growth hormone concentrations decrease to normal levels in 70-90%, with a decrease rate of 10-30% per year. Hypercortisolism is controlled in 50-83% of adults and 80% of children with Cushing's disease, generally in less than 9 months. Hypopituitarism is the most common side effect of pituitary irradiation with an incidence of 13-56%. Long-term overall risk for brain necrosis in a total of 1,388 analyzed patients

  12. Síndromes hormonales paraneoplásicos Paraneoplastic hormonal syndromes

    L. Forga

    2005-08-01

    depend on the secretion of hormonal peptides or their precursors, cytokines and, more rarely, thyroidal hormones and Vitamin D, which act in an endocrine, paracrine or autocrine way. Sometimes, paraneoplastic syndromes can be more serious than the consequences of the primary tumour itself and can precede, develop in parallel, or follow the manifestations of this tumour. It is important to recognise a paraneoplastic hormonal syndrome for several reasons, amongst which we would draw attention to three: 1 It can lead to the diagnosis of a previously undetected, underlying malign or benign neoplasia; 2 It can dominate the clinical picture and thus lead to errors with respect to the origin and type of primary tumour; and 3 It can follow the clinical course of the underlying tumour and thus be useful for monitoring its evolution. The molecular mechanisms responsible for the development of these syndromes are not well-known, but it is believed that they might be inherent to the mutations responsible for the primary tumour or depend on epigenetic factors such as methylation. In this review, we consider the following paraneoplastic hormonal syndromes: malign hypercalcaemia, hyponatraemia (inappropiate secretion of the antidiuretic hormone, ectopic Cushing’s syndrome, ectopic acromegaly, hypoglycaemia due to tumours different from those of the islet cells and paraneoplastic gynaecomastia; we make a brief final reference to other hormones (calcitonin, somatostatin, and VIP.

  13. 垂体微腺瘤的临床内分泌与病理免疫组化类型相关性研究%Micro pituitary adenomas clinical endocrine correlation with pathologic types of immunohistochemical study

    禹亚丽

    2014-01-01

    目的:对垂体微腺瘤的临床症状体征、内分泌与病理免疫组化类型相关性进行探讨。方法选取2010年1月至2012年12月在本院确诊的垂体微腺瘤患者84例,对其进行内分泌、影像学和病理检查,分析检查结果之间的关系。结果患者出现内分泌症状76例(90.47%),免疫组化阳性组内分泌症状发生率[92.59%(75/81)]较免疫组化阴性组[33.33%(1/3)]高,差异具有显著性(χ2=5.92,P<0.05),表明内分泌症状与免疫组化结果相符。血清泌乳素(PRL)阳性组血清PRL增高及闭经、溢乳或月经失调发生率高于PRL阴性组;生长激素(GH)阳性组血清GH增高及巨人症或肢端肥大发生率高于GH阴性组。结论垂体微腺瘤患者的临床表现、内分泌结果与病理免疫组化类型相关,可用来提高垂体微腺瘤的诊断率与治疗率。%Objective To explore the relationship between the clinical symptoms and endocrine of micro pituitary adenomas and pathological immunohistochemical type. Method We had Choosed 84 patients with micro pituitary adenomas from January 2010 to December 2012 in our hospital, then checked the endocrine, imaging and pathological of them, and analysis the relationship between the test results. Result There were 76 cases (90.47%) had endocrine symptoms in all patients, the immunohistochemical positive endocrine symptoms rate was 92.59%(75/81) , this rate was higher than the immunohistochemical negative group which rate was 33.33%(1/3), the difference was statistically signiifcant (χ2=5.92, P<0.05), this indicated that the endocrine symptoms consistent with immunohistochemical results. The increased serum PRL and amenorrhea, spilled milk or incidence of menstrual disorder rate of serum prolactin (PRL) positive group were higher than that of PRL negative groups; increased serum GH and gigantism or acromegaly fertilizer rates of growth hormone (GH) positive group were higher than GH

  14. Diseases associated with hidranitis suppurativa: part 2 of a series on hidradenitis.

    Scheinfeld, Noah

    2013-06-01

    diseases is likely underreported. Pyoderma vegetans has been noted in 2 cases of HS and 4 cases of Inflammatory Bowel Disease (IBD) and is likely a clue to the linkage of the pathology of IBD and HS. Pityriasis rubra pilaris, in particular Type VI related to HIV, has a relationship more commonly with acne conglobata, but with HS also. Single case reports of diseases associated with HS include systemic lupus erythematosus, acromegaly, Down syndrome, Bazex-Dupre´-Christol, and prurtis ani, but these might be coincidences. Pyogenic Arthritis, Pyoderma gangrenosum, and Acne (PAPA Syndrome) and Pyoderma gangrenosum, Acne, and Suppurative Hidradenitis (PASH Syndrome) are pyodermic-arthritic syndromes that are associated with HS. Erythema nodosum and granulomatous lobular mastitis have been reported with HS but the significance of these reports is uncertain. Because of scarring, HS can result in lymphedema including scrotal elephantiasis and verrucous lymphedema. HS is sometimes accompanied by obesity, hypertension, and anemia and can be considered a disease in the spectrum of metabolic syndrome, a skin disease with systemic consequences. HS, like other types of chronic inflammation when long standing in the perianal and perineal areas, can result in squamous cell cancer. A variety of drugs can induce HS. These include lithium, sirolimus, cyclosporine, vemurafenib, and oral contraceptives. Inverse psoriasis or psoriasis vulgaris as a side effect of infliximab therapy may be associated with HS. These associations aside, most cases of HS occur in isolation without coincident morbidity. PMID:24011308

  15. Radiation therapy in the multimodal treatment approach of pituitary adenoma

    Becker, G. [Klinik am Eichert, Goeppingen (Germany). Dept. of Radiooncology and Radiation Therapy; Radiooncologic Univ. Clinic, Tuebingen (Germany); Kocher, M.; Mueller, R.P. [Koeln Univ. (Germany). Clinic of Radiation Therapy; Kortmann, R.D.; Paulsen, F.; Jeremic, B.; Bamberg, M. [Radiooncologic Univ. Clinic, Tuebingen (Germany)

    2002-04-01

    In this paper, literature will be reviewed to assess the role of modern radiotherapy and radiosurgery in the management of pituitary adenomas. Material and Methods: Nowadays, magnetic resonance imaging for the definition of the target volume and a real three-dimensional (3-D) treatment planning with field conformation and the possibility for non-coplanar irradiation has to be recommended. Most groups irradiate these benign tumors with single doses of 1.8-2.0 Gy up to a total dose of 45 Gy or 50.4 Gy in extensive parasellar adenomas. Adenomas are mostly small, well circumscribed lesions, and have, therefore, attracted the use of stereotactically guided high-precision irradiation techniques which allow extreme focussing and provide steep dose gradients with selective treatment of the target and optimal protection of the surrounding brain tissue. Results: Radiation therapy controls tumor growth in 80-98% of patients with non-secreting adenomas and 67-89% for endocrine active tumors. Reviewing the recent literature including endocrine active and non-secreting adenomas, irradiated postoperatively or in case of recurrence the 5-, 10- and 15-year local control rates amount 92%, 89% and 79%. In cases of microprolactinoma primary therapy consists of dopamine agonists. Irradiation should be preferred in patients with macroprolactinomas, when drug therapy and/or surgery failed or for patients medically unsuitable for surgery. Reduction and control of prolactin secretion can be achieved in 44-70% of patients. After radiotherapy in acromegaly patients somatomedin-C and growth hormone concentrations decrease to normal levels in 70-90%, with a decrease rate of 10-30% per year. Hypercortisolism is controlled in 50-83% of adults and 80% of children with Cushing's disease, generally in less than 9 months. Hypopituitarism is the most common side effect of pituitary irradiation with an incidence of 13-56%. Long-term overall risk for brain necrosis in a total of 1,388 analyzed

  16. Tumores hipofisarios en la tercera edad Pituitary tumors in the elderly

    D Moncet

    2010-04-01

    grupo etario.World population has been growing and aging; and pituitary tumors prevalence among the elderly has also increased, summing up to 10-15% of all pituitary tumors. Thirty six elderly patients with pituitary tumors, 20 of them male, were evaluated restrospectively (average age 71 years, range 65-85. A non-functioning adenoma (NFA prevalence was reported in 64% of the cases, followed by acromegaly, prolactinoma and one each of Cushing´s disease, lymphocytic hypophysitis, craniopharyngioma, and Rathke's pouch cyst. Macroadenomas accounted for 89% of tumors and the most frequent presentation was through visual disorders symptoms followed by incidental finding and hormone hypersecretion. During biochemical evaluation, most patients presented different degrees of hypopituitarism, and patients with macroprolactinoma had PRL levels>800ng/ml. Twenty one patients (58% underwent surgery, while 9 underwent radiotherapy. During the 3.6 years follow-up, changes in adenoma size were reported in 3 patients who received no treatment (in 2 cases size increased, whereas in another it decreased. Medical treatment was indicated for those whose pathology persisted or as primary treatment if comorbilities prevented from performing a surgery. No pituitary function improvement was detected after surgery and the appearance of one or more deficiency was noticed in 62% of patients, though visual field did improve in 67%. Definitive diabetes insipidus as a complication was only detected in two patients, and a case of fistula and meningitis was observed. As a conclusion, we reported a NFA prevalence rate smaller than that reported in the world literature, though similar for the other pituitary tumors, and a higher rate of incidental presentation. No complications attributable to patients' age group were detected during surgery. For elderly patients who do not undergo surgery, clinical and imaging follow-up and/or medical treatment could be an option of choice.