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Sample records for acromegaly

  1. Acromegaly

    Directory of Open Access Journals (Sweden)

    Chanson Philippe

    2008-06-01

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

  2. Treatment strategies for acromegaly

    NARCIS (Netherlands)

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

    2005-01-01

    Acromegaly is a chronic debilitating disorder caused by a growth hormone (GH)-producing pituitary adenoma. Active acromegaly is associated with a two- to fourfold increased mortality risk, mainly from cardiovascular disease. Transsphenoidal surgery is considered as the treatment of choice because of

  3. Medical therapy in acromegaly.

    LENUS (Irish Health Repository)

    Sherlock, Mark

    2011-05-01

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

  4. Growth hormone, growth factors, and acromegaly

    Energy Technology Data Exchange (ETDEWEB)

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

    1987-01-01

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

  5. Auditory changes in acromegaly.

    Science.gov (United States)

    Tabur, S; Korkmaz, H; Baysal, E; Hatipoglu, E; Aytac, I; Akarsu, E

    2017-06-01

    The aim of this study is to determine the changes involving auditory system in cases with acromegaly. Otological examinations of 41 cases with acromegaly (uncontrolled n = 22, controlled n = 19) were compared with those of age and gender-matched 24 healthy subjects. Whereas the cases with acromegaly underwent examination with pure tone audiometry (PTA), speech audiometry for speech discrimination (SD), tympanometry, stapedius reflex evaluation and otoacoustic emission tests, the control group did only have otological examination and PTA. Additionally, previously performed paranasal sinus-computed tomography of all cases with acromegaly and control subjects were obtained to measure the length of internal acoustic canal (IAC). PTA values were higher (p acromegaly group was narrower compared to that in control group (p = 0.03 for right ears and p = 0.02 for left ears). When only cases with acromegaly were taken into consideration, PTA values in left ears had positive correlation with growth hormone and insulin-like growth factor-1 levels (r = 0.4, p = 0.02 and r = 0.3, p = 0.03). Of all cases with acromegaly 13 (32%) had hearing loss in at least one ear, 7 (54%) had sensorineural type and 6 (46%) had conductive type hearing loss. Acromegaly may cause certain changes in the auditory system in cases with acromegaly. The changes in the auditory system may be multifactorial causing both conductive and sensorioneural defects.

  6. Medical Therapy of Acromegaly

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    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.

  7. Diabetes in Patients With Acromegaly.

    Science.gov (United States)

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

    2017-02-01

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

  8. Cardiovascular Disease in Acromegaly.

    Science.gov (United States)

    Sharma, Morali D; Nguyen, Anh V; Brown, Spandana; Robbins, Richard J

    2017-01-01

    In patients with acromegaly, chronic excess of growth hormone (GH) and insulin-like growth factor-1 (IGF-1) leads to the development of acromegalic cardiomyopathy. Its main features are biventricular hypertrophy, diastolic dysfunction, and in later stages, systolic dysfunction and congestive heart failure. Surgical and/or pharmacological treatment of acromegaly and control of cardiovascular risk factors help reverse some of these pathophysiologic changes and decrease the high risk of cardiovascular complications.

  9. Acromegaly: A rare disease?

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    Oscar D. Bruno

    2018-04-01

    Full Text Available Acromegaly is generally considered a benign and uncommon disease. However, some recent data bring support to the idea that it is more frequent than previously thought. Besides, acromegaly can significantly shorten the length of life due to its cardiovascular and metabolic complications. Since its clinical signs are insidiously progressive for many years, there is a considerable delay in its detection. Usually, many different specialists have been consulted before reaching diagnosis of acromegaly. Those specialists include cardiologists, pulmonologists, dentists, rheumatologists, and diabetes specialists. Possible means to achieve earlier detection are based on increasing awareness of doctors and the public in general. In this paper, the author analyzes the factors related to delayed diagnosis and the potential ways to ameliorate awareness of the disease with particular attention to screening procedures.

  10. Acromegaly associated with gangliocytoma.

    LENUS (Irish Health Repository)

    Crowley, R K

    2009-09-30

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

  11. Treatment update on acromegaly

    Directory of Open Access Journals (Sweden)

    Tatiana Hotimsky Millner

    2004-12-01

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

  12. Acromegaly : irreversible clinical consequences

    NARCIS (Netherlands)

    Wassenaar, Monica Johanna Elisabeth

    2010-01-01

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

  13. Pachydermoperiostosis Masquerading as Acromegaly.

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    Karimova, Munira M; Halimova, Zamira Yu; Urmanova, Yulduz M; Korbonits, Márta; Cranston, Treena; Grossman, Ashley B

    2017-02-01

    Acromegaly usually is suspected on clinical grounds. Biochemical confirmation is required to optimize therapy, but there are other differential diagnoses. We describe a 24-year-old Uzbek man who presented with many clinical symptoms and signs of apparent acromegaly. On examination, the patient showed a rugose folding of his scalp, with the formation of tender, painful, rough skin folds in the parietal-occipital region, resembling cerebral gyri ( i.e. , cutis verticis gyrate). There was also a thickening and enlargement of the eyelids due to cartilaginous hypertrophy, dystrophic changes of the conjunctiva, and atrophy of the Meibomian glands, with the formation of multiple cysts and granulomas. He perspired excessively. There was thickening of the facial skin, with increased oiliness, increased rugosity, and seborrheic dermatitis. The skin over the hands was thick and apparently fixed to the underlying tissues. However, the patient had a low-normal insulin-like growth factor-1 level. More detailed analysis revealed a family history of relatives with similar problems, and certain features were not in keeping with this diagnosis. The disorder pachydermoperiostosis, or pulmonary hypertrophic osteoarthropathy, was suspected, and next-generation screening confirmed that the patient was homozygous for a pathogenic mutation in the SLCO2A1 gene, c.764G>A (p.Gly255Glu). The condition of pachydermoperiostosis may masquerade as acromegaly but is a genetic disorder, usually autosomal recessive, leading to elevated prostaglandin E2 levels. This is an important, albeit rare, differential diagnosis of acromegaly.

  14. Pregnancy and acromegaly.

    Science.gov (United States)

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

    2017-02-01

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

  15. Radiation techniques for acromegaly

    Directory of Open Access Journals (Sweden)

    Minniti Giuseppe

    2011-12-01

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

  16. Radiation techniques for acromegaly

    International Nuclear Information System (INIS)

    Minniti, Giuseppe; Scaringi, Claudia; Enrici, Riccardo Maurizi

    2011-01-01

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

  17. Endocrine system: acromegaly

    International Nuclear Information System (INIS)

    Linfoot, J.A.

    1980-01-01

    Acromegaly and gigantism represent multisystem diseases resulting from either a primary pituitary tumor or an ill-defined hypothalamo-hypophyseal dysfunction causing somatotropic cell hyperplasia and tumor formation. Clinical manifestations of the disease result from three major processes: (1) excessive growth hormone (HGH) secretion, (2) deficiencies of other pituitary tropic hormones, and (3) local invasion of parasellar neural and vascular structures. The treatment of this disease, and side effects, are discussed

  18. Diabetic retinopathy in acromegaly

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    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.

  19. Gamma Knife Radiosurgery for Acromegaly

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    John D. Rolston

    2012-01-01

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

  20. Current pharmacotherapy for acromegaly: a review

    NARCIS (Netherlands)

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

    2005-01-01

    Acromegaly is associated with considerable morbidity and excess mortality; however, after effective treatment, both morbidity and mortality risks improve. Growth hormone excess in acromegaly can be controlled in many patients by pharmacotherapy alone, and with a combination of transsphenoidal

  1. Diabetic ketoacidosis in a patient with acromegaly

    NARCIS (Netherlands)

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

    2001-01-01

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

  2. Acromegaly: clinical features at diagnosis.

    Science.gov (United States)

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

    2017-02-01

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

  3. Genetics of Gigantism and Acromegaly

    OpenAIRE

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

    2016-01-01

    Gigantism and acromegaly are rare disorders that are caused by excessive GH secretion and/or high levels of its mediator, IGF-1. Gigantism occurs when excess GH or IGF-1 lead to increased linear growth, before the end of puberty and epiphyseal closure. The majority of cases arise from a benign GH-secreting pituitary adenoma, with an incidence of pituitary gigantism and acromegaly of approximately 8 and 11 per million person-years, respectively. Over the past two decades, our increasing unders...

  4. Genetics of Gigantism and Acromegaly

    Science.gov (United States)

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

    2016-01-01

    Gigantism and acromegaly are rare disorders that are caused by excessive GH secretion and/or high levels of its mediator, IGF-1. Gigantism occurs when excess GH or IGF-1 lead to increased linear growth, before the end of puberty and epiphyseal closure. The majority of cases arise from a benign GH-secreting pituitary adenoma, with an incidence of pituitary gigantism and acromegaly of approximately 8 and 11 per million person-years, respectively. Over the past two decades, our increasing understanding of the molecular and genetic etiologies of pituitary gigantism and acromegaly yielded several genetic causes, including multiple endocrine neoplasia type 1 and 4, McCune-Albright syndrome, Carney complex, familial isolated pituitary adenoma, pituitary adenoma association due to defects in familial succinate dehydrogenase genes, and the recently identified X-linked acrogigantism. The early diagnosis of these conditions helps guide early intervention, screening, and genetic counseling of patients and their family members. In this review, we provide a concise and up-to-date discussion on the genetics of gigantism and acromegaly. PMID:27657986

  5. Genetics of gigantism and acromegaly.

    Science.gov (United States)

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

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

  6. Mortality in acromegaly: a metaanalysis

    NARCIS (Netherlands)

    Dekkers, O. M.; Biermasz, N. R.; Pereira, A. M.; Romijn, J. A.; Vandenbroucke, J. P.

    2008-01-01

    Several studies have assessed mortality risk in patients treated for acromegaly. All studies found a mortality that was higher than expected for the general population, but most of these increases were not statistically significant. For this reason, it is not formally established whether mortality

  7. Pegvisomant in acromegaly: an update.

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    Giustina, A; Arnaldi, G; Bogazzi, F; Cannavò, S; Colao, A; De Marinis, L; De Menis, E; Degli Uberti, E; Giorgino, F; Grottoli, S; Lania, A G; Maffei, P; Pivonello, R; Ghigo, E

    2017-06-01

    In 2007, we published an opinion document to review the role of pegvisomant (PEG) in the treatment of acromegaly. Since then, new evidence emerged on the biochemical and clinical effects of PEG and on its long-term efficacy and safety. We here reviewed the emerging aspects of the use of PEG in clinical practice in the light of the most recent literature. The clinical use of PEG is still suboptimal, considering that it remains the most powerful tool to control IGF-I in acromegaly allowing to obtain, with a pharmacological treatment, the most important clinical effects in terms of signs and symptoms, quality of life and comorbidities. The number of patients with acromegaly exposed to PEG worldwide has become quite elevated and the prolonged follow-up allows now to deal quite satisfactorily with many clinical issues including major safety issues, such as the concerns about possible tumour (re)growth under PEG. The positive or neutral impact of PEG on glucose metabolism has been highlighted, and the clinical experience, although limited, with sleep apnoea and pregnancy has been reviewed. Finally, the current concept of somatostatin receptor ligands (SRL) resistance has been addressed, in order to better define the acromegaly patients to whom the PEG option may be offered. PEG increasingly appears to be an effective and safe medical option for many patients not controlled by SRL but its use still needs to be optimized.

  8. Epidemiology of acromegaly in Ecuador.

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

    2016-01-01

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

  9. Clinical Manifestations and Diagnosis of Acromegaly

    OpenAIRE

    Lugo, Gloria; Pena, Lara; Cordido, Fernando

    2012-01-01

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

  10. The Association of Acromegaly and Ankylosing Spondylitis

    OpenAIRE

    Alpaslan Tuzcu; Ayse Dicle Turhanoglu; Mithat Bahçeci; Hatice Öztürkmen Akay; Zülfü Karabulut

    2004-01-01

    Coexistence of acromegaly and anklosing spondylitis had been rarelyreported. Only two case reports were described coexistence of two diseasesin literature. These two diseases have some similar clinical andradiographic features. Calcaneal epin formation, enteshopathy and caudeequina syndrome can be seen both acromegaly and ankylosing spondylitis.Our case had clinical and radiological features of both acromegaly andankylosing spondylitis. Acral enlargement, coarsening of feature,malocclusion, n...

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

    Science.gov (United States)

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

    2017-01-01

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

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

    Science.gov (United States)

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

    2017-10-01

    Acromegaly is a rare disorder caused by chronic growth hormone (GH) hypersecretion. While diagnostic and therapeutic methods have advanced, little information exists on trends in acromegaly characteristics over time. The Liège Acromegaly Survey (LAS) Database , a relational database, is designed to assess the profile of acromegaly patients at diagnosis and during long-term follow-up at multiple treatment centers. The following results were obtained at diagnosis. The study population consisted of 3173 acromegaly patients from ten countries; 54.5% were female. Males were significantly younger at diagnosis than females (43.5 vs 46.4 years; P  3100 patients is the largest international acromegaly database and shows clinically relevant trends in the characteristics of acromegaly at diagnosis. © 2017 The authors.

  13. The surgical treatment of acromegaly.

    Science.gov (United States)

    Buchfelder, Michael; Schlaffer, Sven-Martin

    2017-02-01

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

  14. Acromegaly Presenting with Hemiplegia | Saffer | South African ...

    African Journals Online (AJOL)

    A case of acromegaly presenting with hemiplegia is described. The radiological features, including cerebral angiography, are discussed. Acromegaly is uncommon in the Black population of South Africa. Experience at Baragwanath Hospital (2400 beds) confirms the rarity of the disease, and reference to the South African ...

  15. Urinary growth hormone excretion in acromegaly

    DEFF Research Database (Denmark)

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

    1993-01-01

    The biochemical assessment of disease activity in acromegaly still presents a problem, especially in treated patients with mild clinical symptoms. We therefore examined the diagnostic value of the measurement of urinary growth hormone (GH) excretion in seventy unselected patients with acromegaly...

  16. [Treatment of macroglossia due to acromegaly].

    NARCIS (Netherlands)

    Alons, K.; Berge, S.J.; Rieu, P.N.M.A.; Meijer, G.J.

    2010-01-01

    A 61-years-old woman had macroglossia due to acromegaly with complaints of dyspneu at a lying sleeping position and complaints of speech and dysphagia. At the age of 55 years she was diagnosed with acromegaly induced by a adenoma of the pituitary gland, which had been removed surgically. The

  17. Clinical manifestations and diagnosis of acromegaly.

    Science.gov (United States)

    Lugo, Gloria; Pena, Lara; Cordido, Fernando

    2012-01-01

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

  18. Clinical Manifestations and Diagnosis of Acromegaly

    Directory of Open Access Journals (Sweden)

    Gloria Lugo

    2012-01-01

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

  19. Development of acromegaly in patients with prolactinomas

    DEFF Research Database (Denmark)

    Andersen, Marianne; Hagen, Casper; Frystyk, Jan

    2003-01-01

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

  20. Complications of acromegaly: thyroid and colon.

    Science.gov (United States)

    Tirosh, Amit; Shimon, Ilan

    2017-02-01

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

  1. Megaduodenum in a patient with acromegaly

    Directory of Open Access Journals (Sweden)

    Belinda George

    2012-01-01

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

  2. Pachydermoperiostosis: a rare mimicker of acromegaly

    Directory of Open Access Journals (Sweden)

    Noor Rafhati Adyani Abdullah

    2017-05-01

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

  3. Increased prevalence of regurgitant valvular heart disease in acromegaly

    NARCIS (Netherlands)

    Pereira, Alberto M.; van Thiel, Sjoerd W.; Lindner, Jonathan R.; Roelfsema, Ferdinand; van der Wall, Ernst E.; Morreau, Hans; Smit, Jan W. A.; Romijn, Johannes A.; Bax, Jeroen J.

    2004-01-01

    Cardiac involvement is common in acromegaly, but the prevalence of valvular abnormalities in patients with acromegaly has not been documented and is the topic of this study. In a prospective study design, 40 consecutive patients with acromegaly and 120 control subjects (matched for age, sex,

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

    Directory of Open Access Journals (Sweden)

    Erdal Bodakçi

    2015-06-01

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

  5. Prevalence of diabetes mellitus in patients with acromegaly

    NARCIS (Netherlands)

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

    2014-01-01

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

  6. Acromegaly and McCune-Albright Syndrome

    Science.gov (United States)

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

    2014-01-01

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

  7. Multiple Myeloma in a Patient with Acromegaly

    Directory of Open Access Journals (Sweden)

    Yu Mi Kang

    2015-03-01

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

  8. Pasireotide for the treatment of acromegaly.

    Science.gov (United States)

    Wildemberg, Luiz Eduardo; Gadelha, Mônica R

    2016-01-01

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

  9. Acromegaly

    Science.gov (United States)

    ... glucose Growth hormone and growth hormone suppression test Insulin-like growth factor 1 (IGF-1) Prolactin Spine x-ray MRI of the brain , including the pituitary gland Echocardiogram Other tests may ...

  10. Acromegaly

    Science.gov (United States)

    ... 425. Levy A. Pituitary disease: presentation, diagnosis, and management. Journal of Neurology, Neurosurgery, and Psychiatry . 2004;75:47– ... 30 a.m. to 5 p.m. eastern time, M-F Follow Us NIH… Turning Discovery Into ... Disease Urologic Diseases Endocrine Diseases Diet & Nutrition ...

  11. The Association of Acromegaly and Ankylosing Spondylitis

    Directory of Open Access Journals (Sweden)

    Alpaslan Tuzcu

    2004-01-01

    Full Text Available Coexistence of acromegaly and anklosing spondylitis had been rarelyreported. Only two case reports were described coexistence of two diseasesin literature. These two diseases have some similar clinical andradiographic features. Calcaneal epin formation, enteshopathy and caudeequina syndrome can be seen both acromegaly and ankylosing spondylitis.Our case had clinical and radiological features of both acromegaly andankylosing spondylitis. Acral enlargement, coarsening of feature,malocclusion, non-suppressed growth hormone levels with oral glucosetolerance test and evidence of pituitary adenoma were support diagnose ofacromegaly. Morning stiffness, positive Schober and Moll test, elevatederythrocyte sedimentation rate and grade 4 sacroiletis of the patient lead usto diagnose ankylosing spondylitis at the same time. In this case report, weaim to discuss interesting coexistence of two disease

  12. Pachydermoperiostosis: a rare mimicker of acromegaly.

    Science.gov (United States)

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

    2017-01-01

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

  13. [Acromegaly features in the aging population].

    Science.gov (United States)

    Anoun, Nadia; El Ouahabi, Hanan

    2017-01-01

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

  14. Association between diabetic ketoacidosis and acromegaly

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    Paloma Ocampo

    2018-04-01

    Full Text Available Diabetes mellitus occurs in nearly 10% of patients with acromegaly and is secondary to insulin resistance caused by high levels of growth hormone. Diabetes ketoacidosis has been described as a rare complication of acromegaly, resulting from a relative insulin deficiency caused by growth hormone excess. We described the case of a 38 year-old man who presented to the emergency room with a 6-week history of polydipsia, polyuria, polyphagia and weight loss. He also had nausea, vomiting and abdominal pain from two days before admission. His plasma glucose level was 880 mg/dl, plasma osmolarity 368 mOsm/l, arterial pH 7.06 and serum bicarbonate 8.6 mEq/l. At the clinical examination, he had features of acromegaly. Magnetic resonance imaging showed a pituitary macro adenoma and growth hormone dosages were abnormally high. After tumor removal, plasma glucose levels became normal. This case shows the rare association between diabetic ketoacidosis and acromegaly. Surgery, in this case, was the definite modality of treatment.

  15. An update on the treatment of acromegaly

    Directory of Open Access Journals (Sweden)

    Edling KL

    2013-02-01

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

  16. Current therapies and mortality in acromegaly.

    Science.gov (United States)

    Găloiu, S; Poiană, C

    2015-01-01

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

  17. Acromegaly without acral changes: A rare presentation

    Directory of Open Access Journals (Sweden)

    Nilanjan Sengupta

    2012-01-01

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

  18. Adiponectin and Cardiac Hypertrophy in Acromegaly.

    Science.gov (United States)

    Gurbulak, Sabriye; Akin, Fulya; Yerlikaya, Emrah; Yaylali, Guzin F; Topsakal, Senay; Tanriverdi, Halil; Akdag, Beyza; Kaptanoglu, Bunyamin

    2016-01-01

    Adiponectin is an adipocytes-derived hormone which has been shown to possess insulin-sensitizing, antiatherogenic, and anti-inflammatory properties. In acromegaly, the data on adiponectin is contradictory. The relationship between adiponectin levels and cardiac parameters has not been studied. The aim of this study was to find out how adiponectin levels were affected in acromegalic patients and the relationship between adiponectin levels and cardiac parameters. We included 30 subjects (15 male, 15 female), diagnosed with acromegaly and 30 healthy (10 male, 20 female) subjects. Serum glucose, insulin, GH, IGF-1 and adiponectin levels were obtained and the insulin resistance of the subjects was calculated. Echocardiographic studies of the subjects were performed. We determined that adiponectin levels were significantly higher in the acromegalic group than the control group. In the acromegalic group, there was no statistically significant relation between serum adiponectin and growth hormone (GH), or insulin-like growth factor-1 (IGF-1) levels (p = 0.3, p = 0.1). We demonstrated that cardiac function and structure are affected by acromegaly. IVST, PWT, LVMI, E/A ratio, DT, ET, IVRT, VPR, and LVESV values were increased and the results were statistically significant. In the acromegalic group, adiponectin levels were positively related with left ventricle mass index (LVMI) but this correlation was found to be statistically weak (p = 0.03). In our study, there was a positive correlation between VAI and LVM. We also could not find any correlation between VAI and adiponectin levels. Although insulin resistance and high insulin levels occur in active acromegaly patients, adiponectin levels were higher in our study as a consequence of GH lowering therapies. Our study showed that adiponectin levels may be an indicator of the cardiac involvement acromegaly. However, the usage of serum adiponectin levels in acromegalic patients as an indicator of cardiac involvement should be

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

    Science.gov (United States)

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

    2017-01-01

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

  20. Acromegaly and aging: a comparative cross-sectional study.

    Science.gov (United States)

    Hatipoglu, Esra; Yuruyen, Mehmet; Keskin, Ela; Yavuzer, Hakan; Niyazoglu, Mutlu; Doventas, Alper; Erdincler, Deniz Suna; Beger, Tanju; Kadioglu, Pinar; Gundogdu, Sadi

    2015-02-01

    Cognitive and functional geriatric assessment may change in acromegaly. Herein we aimed to determine at which points geriatric assessment of the cases with acromegaly differs from that of general elderly population. In this comparative cross-sectional study, a total of 30 cases with acromegaly (controlled n = 14, uncontrolled n = 16) and 30 gender and body-mass index-matched cases without acromegaly (control group, CG) above 60 years old were included. Cognitive functions were evaluated on the basis of the mini-mental state exam (MMSE). Affective status was determined using the geriatric depression scale. Activities of daily living (ADL) were ranked according to the Barthel index while instrumental activities of daily living (IADL) were graded on the basis of the Lawton scale. Nutritional status was evaluated using the mini-nutritional assessment (MNA). Body composition was measured through bioimpedance analysis. Functional mobility was determined using the Timed Up and Go test (TUG) and muscle strength with the handgrip strength test. Scores on the MMSE were significantly lower in the elderly cases with acromegaly than in the cases without acromegaly (p < 0.001). Dementia was more frequent in the acromegaly group than in the CG (p = 0.04). Total MNA scores were significantly lower in cases with acromegaly than in the CG (p = 0.006). More subjects in the acromegaly group (33%) were at greater risk of malnutrition than in the CG (3%) (p = 0.003). There was greater moderate functional impairment based on Barthel ADL in the acromegaly group than in the CG (p = 0.04). Acromegaly may impair cognitive functions, functional mobility and instrumental daily living activities in the geriatric population. With acromegaly, the risk of malnutrition may also increase. Copyright © 2014 Elsevier Ltd. All rights reserved.

  1. Corneal endothelial cell density and morphology in patients with acromegaly.

    Science.gov (United States)

    Hatipoglu, Esra; Arici, Ceyhun; Arslan, Osman Sevki; Dikkaya, Funda; Sultan, Pinar; Kadioglu, Pinar; Gundogdu, Sadi

    2014-12-01

    Acromegaly has various impacts on many organs. The ophthalmologic effects of acromegaly have not yet been investigated in detail. The aim of the current study was to evaluate qualitative and quantitative changes in corneal endothelial cells and central corneal thickness (CCT) of the patients with acromegaly. In this prospective, cross-sectional study, 128 eyes of 64 patients with acromegaly (female/male=40/24) and 208 eyes of 104 age and gender-matched healthy volunteers (female/male=69/35) were included. Endothelial cell density (ECD), cellular area (CA), coefficient of variation (CV) in cell size, percentage of hexagonal cells, and CCT were measured in patients with acromegaly and in healthy volunteers using the noncontact specular microscopy (SP-3000P: Topcon Corporation, Tokyo, Japan). ECD and CA were lower in cases with acromegaly than in controls (ECD in acromegaly: 2615.65 cell/mm(2) and in controls: 2700.35 cell/mm(2); p=0.002. CA in acromegaly: 382.30μm(2) and in controls: 400.30μm(2); p=0.02). In the entire group with acromegaly, the time elapsed since diagnosis was positively correlated with CA and was negatively correlated with ECD (r=+0.39, p=0.001 and r=-0.42, p=0.001). The endothelial layer of the cornea may be under risk of impairment with prolonged disease duration in acromegaly. Consistency of the corneal endothelium should be also sought during long-term follow-up of the cases with acromegaly. Copyright © 2014 Elsevier Ltd. All rights reserved.

  2. MRI of pituitary adenomas in acromegaly

    International Nuclear Information System (INIS)

    Marro, B.; Zouaoui, A.; Sahel, M.; Crozat, N.; Gerber, S.; Sourour, N.; Sag, K.; Marsault, C.

    1997-01-01

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

  3. Acromegaly said to respond to proton therapy

    Energy Technology Data Exchange (ETDEWEB)

    Raymond, C.A.

    1988-02-12

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

  4. Treatment of acromegaly by external irradiation

    International Nuclear Information System (INIS)

    Macleod, A.F.; Clarke, D.G.; Pambakian, H.; Lowy, C.; Sonksen, P.H.; Collins, C.D.

    1989-01-01

    Despite hypophysectomy as the primary treatment in acromegaly, external radiotherapy maintains a role as a relatively slow but effective therapy for inadequately treated patients or those unsuitable for operation. Over the last 25 years the authors' regimen has differed from published series in that they give a larger dose per fraction, with fewer treatments. They have analysed the efficacy and side-effects of this regimen in 27 subjects with acromegaly. Growth hormone levels have fallen by, on average, 27% per year in the first five years, 83% of subjects achieving a basal growth hormone of <10mU/1. The acute and chronic side-effects of irradiation are discussed, including the relevance of estimates of biological potency, for example Time Dose Fraction (TDF). One patient suffered visual loss that was most likely to be secondary to the radiotherapy. Histological appearances of the pituitary fossa in five subjects previously treated with radiotherapy are reported. (author)

  5. Treatment of acromegaly by external irradiation

    Energy Technology Data Exchange (ETDEWEB)

    Macleod, A.F.; Clarke, D.G.; Pambakian, H.; Lowy, C.; Sonksen, P.H.; Collins, C.D. (Saint Thomas' Hospital, London (UK))

    1989-03-01

    Despite hypophysectomy as the primary treatment in acromegaly, external radiotherapy maintains a role as a relatively slow but effective therapy for inadequately treated patients or those unsuitable for operation. Over the last 25 years the authors' regimen has differed from published series in that they give a larger dose per fraction, with fewer treatments. They have analysed the efficacy and side-effects of this regimen in 27 subjects with acromegaly. Growth hormone levels have fallen by, on average, 27% per year in the first five years, 83% of subjects achieving a basal growth hormone of <10mU/1. The acute and chronic side-effects of irradiation are discussed, including the relevance of estimates of biological potency, for example Time Dose Fraction (TDF). One patient suffered visual loss that was most likely to be secondary to the radiotherapy. Histological appearances of the pituitary fossa in five subjects previously treated with radiotherapy are reported. (author).

  6. Acromegaly said to respond to proton therapy

    International Nuclear Information System (INIS)

    Raymond, C.A.

    1988-01-01

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

  7. MRI of pituitary adenomas in acromegaly

    Energy Technology Data Exchange (ETDEWEB)

    Marro, B. [Service de Neuroradiologie, Batiment Babinsky, Hopital Salpetriere, 47, Bd de l`Hopital, F-75013 Paris (France); Zouaoui, A. [Service de Neuroradiologie, Batiment Babinsky, Hopital Salpetriere, 47, Bd de l`Hopital, F-75013 Paris (France); Sahel, M. [Service de Neuroradiologie, Batiment Babinsky, Hopital Salpetriere, 47, Bd de l`Hopital, F-75013 Paris (France); Crozat, N. [Service de Neuroradiologie, Batiment Babinsky, Hopital Salpetriere, 47, Bd de l`Hopital, F-75013 Paris (France); Gerber, S. [Service de Neuroradiologie, Batiment Babinsky, Hopital Salpetriere, 47, Bd de l`Hopital, F-75013 Paris (France); Sourour, N. [Service de Neuroradiologie, Batiment Babinsky, Hopital Salpetriere, 47, Bd de l`Hopital, F-75013 Paris (France); Sag, K. [Service de Neuroradiologie, Batiment Babinsky, Hopital Salpetriere, 47, Bd de l`Hopital, F-75013 Paris (France); Marsault, C. [Service de Neuroradiologie, Batiment Babinsky, Hopital Salpetriere, 47, Bd de l`Hopital, F-75013 Paris (France)

    1997-06-01

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

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

    International Nuclear Information System (INIS)

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

    2015-01-01

    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

  9. A Structural and Functional Acromegaly Classification

    Science.gov (United States)

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

    2015-01-01

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

  10. Glioblastoma multiforme after radiotherapy for acromegaly

    Energy Technology Data Exchange (ETDEWEB)

    Piatt, J.H. Jr.; Blue, J.M.; Schold, S.C. Jr.; Burger, P.C.

    1983-07-01

    A case of glioblastoma multiforme that occurred 14 years after radiotherapy for acromegaly is presented. The striking correspondence between the anatomy of the tumor and the geometry of the radiation ports is suggestive of a causal relationship. Previously reported cases of radiation-associated glioma are reviewed, and a brief appraisal of the evidence for induction of these lesions by radiation is presented. The differentiation of radiation-associated neoplasms from radionecrosis is also discussed.

  11. Pachydermoperiostosis: a rare mimicker of acromegaly

    OpenAIRE

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

    2017-01-01

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

  12. Treatment of acromegaly by yttrium implantation

    Energy Technology Data Exchange (ETDEWEB)

    Hibbert, J.; Shaheen, O.H.

    1977-01-01

    Yttrium implantation is one of the many ways of treating acromegaly. The advantages are the minor nature of the procedure and the fact that pituitary replacement is not as commonly required as after hypophysectomy. Thus in young female patients menstruation may be resumed following treatment and pregnancy has occurred. The procedure is not as free from complications as external irradiation but the response is more satisfactory.

  13. Glioblastoma multiforme after radiotherapy for acromegaly

    International Nuclear Information System (INIS)

    Piatt, J.H. Jr.; Blue, J.M.; Schold, S.C. Jr.; Burger, P.C.

    1983-01-01

    A case of glioblastoma multiforme that occurred 14 years after radiotherapy for acromegaly is presented. The striking correspondence between the anatomy of the tumor and the geometry of the radiation ports is suggestive of a causal relationship. Previously reported cases of radiation-associated glioma are reviewed, and a brief appraisal of the evidence for induction of these lesions by radiation is presented. The differentiation of radiation-associated neoplasms from radionecrosis is also discussed

  14. Novel Somatostatin Receptor Ligands Therapies for Acromegaly

    Directory of Open Access Journals (Sweden)

    Rosa Maria Paragliola

    2018-03-01

    Full Text Available Surgery is considered the treatment of choice in acromegaly, but patients with persistent disease after surgery or in whom surgery cannot be considered require medical therapy. Somatostatin receptor ligands (SRLs octreotide (OCT, lanreotide, and the more recently approved pasireotide, characterized by a broader receptor ligand binding profile, are considered the mainstay in the medical management of acromegaly. However, in the attempt to offer a more efficacious and better tolerated medical approach, recent research has been aimed to override some limitations related to the use of currently approved drugs and novel SRLs therapies, with potential attractive features, have been proposed. These include both new formulation of older molecules and new molecules. Novel OCT formulations are aimed in particular to improve patients’ compliance and to reduce injection discomfort. They include an investigational ready-to-use subcutaneous depot OCT formulation (CAM2029, delivered via prefilled syringes and oral OCT that uses a “transient permeability enhancer” technology, which allows for OCT oral absorption. Another new delivery system is a long-lasting OCT implant (VP-003, which provide stable doses of OCT throughout a period of several months. Finally, a new SRL DG3173 (somatoprim seems to be more selective for GH secretion, suggesting possible advantages in the presence of hyperglycemia or diabetes. How much these innovations will actually be beneficial to acromegaly patients in real clinical practice remains to be seen.

  15. Pulmonary epidermoid carcinoma in a patient with acromegaly: a ...

    African Journals Online (AJOL)

    A 56-years-old woman was referred to our unit for partially treated acromegaly. She had a high level of insulin growth factor. She did not complain of any pulmonary symptoms and was a non-smoker. Physical examination revealed clinical features of acromegaly. She had a 13 mm pituitary adenoma and was proposed for ...

  16. Multi-modal management of acromegaly: a value perspective.

    Science.gov (United States)

    Kimmell, Kristopher T; Weil, Robert J; Marko, Nicholas F

    2015-10-01

    The Acromegaly Consensus Group recently released updated guidelines for medical management of acromegaly patients. We subjected these guidelines to a cost analysis. We conducted a cost analysis of the recommendations based on published efficacy rates as well as publicly available cost data. The results were compared to findings from a previously reported comparative effectiveness analysis of acromegaly treatments. Using decision tree software, two models were created based on the Acromegaly Consensus Group's recommendations and the comparative effectiveness analysis. The decision tree for the Consensus Group's recommendations was subjected to multi-way tornado analysis to identify variables that most impacted the value analysis of the decision tree. The value analysis confirmed the Consensus Group's recommendations of somatostatin analogs as first line therapy for medical management. Our model also demonstrated significant value in using dopamine agonist agents as upfront therapy as well. Sensitivity analysis identified the cost of somatostatin analogs and growth hormone receptor antagonists as having the most significant impact on the cost effectiveness of medical therapies. Our analysis confirmed the value of surgery as first-line therapy for patients with surgically accessible lesions. Surgery provides the greatest value for management of patients with acromegaly. However, in accordance with the Acromegaly Consensus Group's recent recommendations, somatostatin analogs provide the greatest value and should be used as first-line therapy for patients who cannot be managed surgically. At present, the substantial cost is the most significant negative factor in the value of medical therapies for acromegaly.

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

    Science.gov (United States)

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

    2017-02-01

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

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

    Science.gov (United States)

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

    2016-01-01

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

  19. Outcome in acromegaly: A retrospective analysis

    Directory of Open Access Journals (Sweden)

    Debmalya Sanyal

    2012-01-01

    Full Text Available Introduction: Many of the treatment modalities recommended for acromegaly are either too expensive or not available in large parts of India. There is a dearth of treatment and outcome data in Indian patients. Aims and Objectives: The purpose of this study was to analyze the treatment modalities used and the respective outcomes which include remission, recurrence, hypopituitarism, other complications, and mortality. Materials and Methods: This is a retrospective data analysis of 15 acromegaly patients treated at a tertiary care hospital in eastern India. A remission criteria of nadir growth hormone level <1 μg/dl after Oral Glucose tolerance test (OGTT and normal age related IGF-1 levels was used. Results: All patients (100% had macroadenomas. Surgery could not be done in five (33%; three (19.8% refused, two (13.2% had comorbidities. Transsphenoidal surgery (TSS achieved remission in four out of ten (40%. Conventional radiotherapy (CRT failed in all five patients and caused hypopituitarisn in three (60%. Cabergoline (CAB either alone or following surgery achieved remission in one out of four (25% though symptomatic relief and tolerability were remarkable. One patient (7% had pituitary apoplexy with remission, two patients (14.3% died due to CVA. Conclusions: TSS remains the treatment of choice in acromegaly, though in macroadenomas the success is limited. A sizeable proportion of patients refuse or are unfit for surgery. As most of the recommended options are very costly or unavailable, alternative treatment options generally used are CRT or CAB which have limited efficacy. Incidence of hypopituitarism, following CRT is very high.

  20. Outcome of Endoscopic Transsphenoidal Surgery for Acromegaly.

    Science.gov (United States)

    Kim, Jung Hee; Hur, Kyu Yeon; Lee, Jung Hyun; Lee, Ji Hyun; Se, Young-Bem; Kim, Hey In; Lee, Seung Hoon; Nam, Do-Hyun; Kim, Seong Yeon; Kim, Kwang-Won; Kong, Doo-Sik; Kim, Yong Hwy

    2017-08-01

    Endoscopic transsphenoidal surgery has recently been introduced in pituitary surgery. We investigated outcomes and complications of endoscopic surgery in 2 referral centers in Korea. We enrolled 134 patients with acromegaly (microadenomas, n = 15; macroadenomas, n = 119) who underwent endoscopic transsphenoidal surgery at Seoul National University Hospital (n = 74) and Samsung Medical Center (n = 60) between January 2009 and March 2016. Remission was defined as having a normal insulin-like growth factor-1 and a suppressed growth hormone (GH) surgery, normal pituitary function was maintained in 34 patients (25.4%). Sixty-four patients (47.7%) presented complete (n = 59, 44.0%) or incomplete (n = 5, 3.7%) recovery of pituitary function. Hypopituitarism persisted in 20 patients (14.9%) and worsened in 16 patients (11.9%). Postoperatively, transient diabetes insipidus was reported in 52 patients (38.8%) but only persisted in 2 patients (1.5%). Other postoperative complications were epistaxis (n = 2), cerebral fluid leakage (n = 4), infection (n = 1), and intracerebral hemorrhage (n = 1). Endoscopic transsphenoidal surgery for acromegaly presented high remission rates and a low incidence of endocrine deficits and complications. Regardless of surgical techniques, invasive pituitary tumors were associated with poor outcome. Copyright © 2017 Elsevier Inc. All rights reserved.

  1. Diabetic Ketoacidosis in a Patient with Acromegaly

    Directory of Open Access Journals (Sweden)

    Yen-Ling Chen

    2007-09-01

    Full Text Available Diabetes mellitus develops in about 10% of acromegalic patients, usually secondary to insulin resistance caused by growth hormone excess. Diabetic ketoacidosis is a result of relative insulin deficiency and is a rare feature of acromegaly. Here, we present one case of this disorder. A 57-year-old man came to the emergency room due to 2 weeks of dizziness. He also had polyuria, polydipsia, nausea, diplopia, blurred vision and dysarthria. His plasma glucose level was 32.06 mmol/L, plasma osmolarity was 322 mOsm/L, arterial pH was 7.30, level of bicarbonates was 18 mmol/L, urine ketones was 4+, and HbA1c was 14.1%. No specific cause for the development of this metabolic derangement could be found. He displayed clinical features of acromegaly during admission, which was confirmed by an elevated growth hormone level and pituitary macroadenoma shown on magnetic resonance imaging. The patient underwent total transsphe-noid tumor removal 2 weeks later; plasma glucose levels became normal thereafter.

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

    Directory of Open Access Journals (Sweden)

    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.

  3. Current and future medical treatments for patients with acromegaly.

    Science.gov (United States)

    Maffezzoni, Filippo; Formenti, Anna Maria; Mazziotti, Gherardo; Frara, Stefano; Giustina, Andrea

    2016-08-01

    Acromegaly is a relatively rare condition of growth hormone (GH) excess associated with significant morbidity and, when left untreated, high mortality. Therapy for acromegaly is targeted at decreasing GH and insulin-like growth hormone 1 levels, ameliorating patients' symptoms and decreasing any local compressive effects of the pituitary adenoma. The therapeutic options for acromegaly include surgery, medical therapies (such as dopamine agonists, somatostatin receptor ligands and the GH receptor antagonist pegvisomant) and radiotherapy. However, despite all these treatments option, approximately 50% of patients are not adequately controlled. In this paper, the authors discuss: 1) efficacy and safety of current medical therapy 2) the efficacy and safety of the new multireceptor-targeted somatostatin ligand pasireotide 3) medical treatments currently under clinical investigation (oral octreotide, ITF2984, ATL1103), and 4) preliminary data on the use of new injectable and transdermal/transmucosal formulations of octreotide. This expert opinion supports the need for new therapeutic agents and modalities for patients with acromegaly.

  4. Acromegaly Presenting as Cardiac Failure - A Case Report

    Directory of Open Access Journals (Sweden)

    Shohael Mahmud Arafat

    2011-09-01

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

  5. Characteristics of Acromegaly in Korea with a Literature Review

    Directory of Open Access Journals (Sweden)

    Jae Won Hong

    2013-09-01

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

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

    Science.gov (United States)

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

    2017-05-01

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

  7. Bone mineral density of lumbar spine and femur in acromegaly

    International Nuclear Information System (INIS)

    Huebsch, P.; Kotzmann, H.; Svoboda, T.; Kainberger, F.M.; Bankier, A.; Seidl, G.

    1993-01-01

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

  8. Growth hormone deficiency in treated acromegaly and active Cushing's syndrome.

    Science.gov (United States)

    Formenti, Anna Maria; Maffezzoni, Filippo; Doga, Mauro; Mazziotti, Gherardo; Giustina, Andrea

    2017-02-01

    Growth hormone deficiency (GHD) in adults is characterized by reduced quality of life and physical fitness, skeletal fragility, increased weight and cardiovascular risk. It may be found in (over-) treated acromegaly as well as in active Cushing's syndrome. Hypopituitarism may develop in patients after definitive treatment of acromegaly, although the exact prevalence of GHD in this population is still uncertain because of limited awareness, and scarce and conflicting data so far available. Because GHD associated with acromegaly and Cushing's syndrome may yield adverse consequences on similar target systems, the final outcomes of some complications of both acromegaly and Cushing's syndrome may be further affected by the occurrence of GHD. It is still largely unknown, however, whether GHD in patients with post-acromegaly or active Cushing's syndrome (e.g. pharmacologic glucocorticoid treatment) may benefit from GH replacement. We review the diagnostic, clinical and therapeutic aspects of GHD in adults treated for acromegaly and in those with active Cushing's syndrome. Copyright © 2017 Elsevier Ltd. All rights reserved.

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

    DEFF Research Database (Denmark)

    Roug, Anne Stidsholt; Rasmussen, Åse Krogh; Juhler, M

    2010-01-01

    To evaluate the effect of fractionated stereotactic radiotherapy (FSRT) in acromegaly in a retrospective analysis.......To evaluate the effect of fractionated stereotactic radiotherapy (FSRT) in acromegaly in a retrospective analysis....

  10. Diabetic ketoacidosis in acromegaly; a rare complication precipitated by corticosteroid use.

    Science.gov (United States)

    Weiss, Jeremy; Wood, Anna J; Zajac, Jeffrey D; Grossmann, Mathis; Andrikopoulos, Sofianos; Ekinci, Elif I

    2017-12-01

    Diabetic ketoacidosis has been described in the literature as a rare possible initial presentation of acromegaly before a diagnosis of acromegaly is eventually made. Indeed, diabetic ketoacidosis is a recognised complication of acromegaly. There are a number of factors that can predispose patients with acromegaly to diabetes as well as to diabetic ketoacidosis. These include high levels of growth hormone and insulin-like growth factor 1 in acromegaly and the effect on glycaemia by medications used in the management of acromegaly. Ketoacidosis has been described in patients with acromegaly even without the presence of an underlying autoimmune diabetes. Patients with acromegaly and ketoacidosis often respond to treatment and may not require long-term insulin. Copyright © 2017. Published by Elsevier B.V.

  11. Gamma-Knife surgery (GKS) in patients with acromegaly: safety and efficacy

    International Nuclear Information System (INIS)

    Katz, D.; Miragaya, K.; Tenca, E.; Margni, A.; Artes, C.; Antico, J.

    2007-01-01

    The acromegaly is associated with increased morbidity and mortality than the general population. Since the surgical and pharmacological treatment for acromegaly have specific limitations, the GKS has been used as a therapeutic option in selected patients. The object is to evaluate the efficacy and safety of GKS in patients with acromegaly [es

  12. Gammaknife radiosurgery in patients with acromegaly.

    Science.gov (United States)

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

    2011-12-01

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

  13. Gene Expression Signature in Adipose Tissue of Acromegaly Patients

    Science.gov (United States)

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

    2015-01-01

    To study the effect of chronic excess growth hormone on adipose tissue, we performed RNA sequencing in adipose tissue biopsies from patients with acromegaly (n = 7) or non-functioning pituitary adenomas (n = 11). The patients underwent clinical and metabolic profiling including assessment of HOMA-IR. Explants of adipose tissue were assayed ex vivo for lipolysis and ceramide levels. Patients with acromegaly had higher glucose, higher insulin levels and higher HOMA-IR score. We observed several previously reported transcriptional changes (IGF1, IGFBP3, CISH, SOCS2) that are known to be induced by GH/IGF-1 in liver but are also induced in adipose tissue. We also identified several novel transcriptional changes, some of which may be important for GH/IGF responses (PTPN3 and PTPN4) and the effects of acromegaly on growth and proliferation. Several differentially expressed transcripts may be important in GH/IGF-1-induced metabolic changes. Specifically, induction of LPL, ABHD5, and NRIP1 can contribute to enhanced lipolysis and may explain the elevated adipose tissue lipolysis in acromegalic patients. Higher expression of TCF7L2 and the fatty acid desaturases FADS1, FADS2 and SCD could contribute to insulin resistance. Ceramides were not different between the two groups. In summary, we have identified the acromegaly gene expression signature in human adipose tissue. The significance of altered expression of specific transcripts will enhance our understanding of the metabolic and proliferative changes associated with acromegaly. PMID:26087292

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

    Science.gov (United States)

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

    2017-07-01

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

  15. Acromegaly treatment in Romania. How close are we to disease control?

    Science.gov (United States)

    Niculescu, Dan Alexandru; Baciu, Ionela Florina; Capatina, Cristina; Galoiu, Simona Andreea; Gheorghiu, Monica Livia; Radian, Serban; Trifanescu, Raluca Alexandra; Caragheorgheopol, Andra; Coculescu, Mihail; Poiana, Catalina

    2017-01-01

    In Romania, no nationwide data for acromegaly treatment and control rate are available. Our objective was to assess the acromegaly control rate in a tertiary referral centre, which covers an important part of Romanian territory and population of patients with acromegaly. We reviewed the records of all 164 patients (49 males and 115 females; median age 55 [47, 63.5] years) with newly or previously diagnosed acromegaly, who have been assessed at least once in our tertiary referral centre between January 1, 2012 and March 31, 2016. This sample represents 13.6% of the total expected 1200 Romanian patients with acromegaly and covers 82.9% of the counties in Romania. Control of acromegaly was defined as a random serum growth hormone (GH) acromegaly benefits from the whole spectrum of therapeutic interventions. However, the control rate remains disappointing.

  16. Restrictive extraocular myopathy: A presenting feature of acromegaly

    Directory of Open Access Journals (Sweden)

    Steven Heireman

    2011-01-01

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

  17. Craniofacial and pharyngeal airway morphology in patients with acromegaly.

    Science.gov (United States)

    Balos Tuncer, Burcu; Canigur Bavbek, Nehir; Ozkan, Cigdem; Tuncer, Cumhur; Eroglu Altinova, Alev; Gungor, Kahraman; Akturk, Mujde; Balos Toruner, Fusun

    2015-08-01

    The aim of this study was to assess differences in craniofacial characteristics, upper spine and pharyngeal airway morphology in patients with acromegaly compared with healthy individuals. Twenty-one patients with acromegaly were compared with 22 controls by linear and angular measurements on cephalograms. The differences between the mean values of cephalometric parameters were analyzed with Mann-Whitney U-test. With respect to controls, anterior (pacromegaly. Craniofacial changes were predominantly found in the frontal bone (pacromegaly exhibited diminished dimensions at nasal (pacromegaly. Current results point to the importance of the reduced airway dimensions and that dentists and/or orthodontists should be aware of the cranial or dental abnormalities in patients with acromegaly.

  18. Malignant ventricular tachycardia in acromegaly: a case report

    Directory of Open Access Journals (Sweden)

    Zhe An

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

  19. Late presentation of acromegaly in medically controlled prolactinoma patients

    Directory of Open Access Journals (Sweden)

    Ekaterina Manuylova

    2016-10-01

    Full Text Available Co-secretion of growth hormone (GH and prolactin (PRL from a single pituitary adenoma is common. In fact, up to 25% of patients with acromegaly may have PRL co-secretion. The prevalence of acromegaly among patients with a newly diagnosed prolactinoma is unknown. Given the possibility of mixed GH and PRL co-secretion, the current recommendation is to obtain an insulin-like growth factor-1 (IGF-1 in patients with prolactinoma at the initial diagnosis. Long-term follow-up of IGF-1 is not routinely done. Here, we report two cases of well-controlled prolactinoma on dopamine agonists with the development of acromegaly 10–20 years after the initial diagnoses. In both patients, a mixed PRL/GH-cosecreting adenoma was confirmed on the pathology examination after transsphenoidal surgery (TSS. Therefore, periodic routine measurements of IGF-1 should be considered regardless of the duration and biochemical control of prolactinoma.

  20. Pasireotide: a novel treatment for patients with acromegaly

    Science.gov (United States)

    Cuevas-Ramos, Daniel; Fleseriu, Maria

    2016-01-01

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

  1. Decreased 133Xe clearance in the proximal femur in acromegaly

    International Nuclear Information System (INIS)

    Kapitola, J.; Marek, J.; Jahoda, I.; Vilimovska, D.

    1986-01-01

    Using the 133 Xe tissue clearance method, the blood flow in the greater trochanter of the femur was studied in 30 patients with acromegaly. Both the washout rate constant (k 2 ) and blood flow (P 2 ) values are significantly decreased in acromegaly (p < 0.01). There is a significant negative correlation (r = -0.42, p < 0.05) between the flow values and mean daily concentrations of growth hormone in serum of acromegalic patients. The observation is presented as a preliminary evidence of a possible influence of growth hormone on the blood flow in bone. (author)

  2. Coagulation parameters and platelet function analysis in patients with acromegaly.

    Science.gov (United States)

    Colak, A; Yılmaz, H; Temel, Y; Demirpence, M; Simsek, N; Karademirci, İ; Bozkurt, U; Yasar, E

    2016-01-01

    Acromegaly is associated with increased cardiovascular morbidity and mortality. The data about the evaluation of coagulation and fibrinolysis in acromegalic patients are very limited and to our knowledge, platelet function analysis has never been investigated. So, we aimed to investigate the levels of protein C, protein S, fibrinogen, antithrombin 3 and platelet function analysis in patients with acromegaly. Thirty-nine patients with active acromegaly and 35 healthy subjects were included in the study. Plasma glucose and lipid profile, fibrinogen levels, GH and IGF-1 levels and protein C, protein S and antithrombin III activities were measured in all study subjects. Also, platelet function analysis was evaluated with collagen/ADP and collagen-epinephrine-closure times. Demographic characteristics of the patient and the control were similar. As expected, fasting blood glucose levels and serum GH and IGF-1 levels were significantly higher in the patient group compared with the control group (pglc: 0.002, pGH: 0.006, pIGF-1: 0.001, respectively). But lipid parameters were similar between the two groups. While serum fibrinogen and antithrombin III levels were found to be significantly higher in acromegaly group (p fibrinogen: 0.005 and pantithrombin III: 0.001), protein S and protein C activity values were significantly lower in the patient group (p protein S: 0.001, p protein C: 0.001). Also significantly enhanced platelet function (measured by collagen/ADP- and collagen/epinephrine-closure times) was demonstrated in acromegaly (p col-ADP: 0.002, p col-epinephrine: 0.002). The results did not change, when we excluded six patients with type 2 diabetes in the acromegaly group. There was a negative correlation between serum GH levels and protein S (r: -0.25, p: 0.04)) and protein C (r: -0.26, p: 0.04) values. Likewise, there was a negative correlation between IGF-1 levels and protein C values (r: -0.39, p: 0.002), protein S values (r: -0.39, p: 0.001), collagen

  3. Results of Gamma Knife Radiosurgery in Acromegaly

    Directory of Open Access Journals (Sweden)

    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.

  4. Uncontrolled acromegaly is associated with progressive mitral valvular regurgitation

    NARCIS (Netherlands)

    van der Klaauw, A. A.; Bax, J. J.; Roelfsema, F.; Bleeker, G. B.; Holman, E. R.; Corssmit, E. P. M.; van der Wall, E. E.; Smit, J. W. A.; Romijn, J. A.; Pereira, A. M.

    2006-01-01

    Recent cross-sectional studies have documented an association between acromegaly and regurgitant valvular heart disease. The aim of this study was to evaluate the change in prevalence of valvular heart disease in relation to the clinical activity, because the natural history of valvular changes in

  5. Non Piluitary Surgery in a patient with Acromegaly complicated by ...

    African Journals Online (AJOL)

    The anaesthetic management of patients with acromegaly, a rare clinical syndrome, for urgent non pituitary surgery has little documentation in the literature. We report such a case further complicated by dilated cardiomyopathy. The anaethetic challenges in the face of limited resources are highlighted. Keywords: ...

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

    Science.gov (United States)

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

    2017-07-01

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

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

    Directory of Open Access Journals (Sweden)

    Murray B. Gordon

    2016-01-01

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

  8. Mortality in acromegaly: a 20-year follow-up study.

    Science.gov (United States)

    Ritvonen, Elina; Löyttyniemi, Eliisa; Jaatinen, Pia; Ebeling, Tapani; Moilanen, Leena; Nuutila, Pirjo; Kauppinen-Mäkelin, Ritva; Schalin-Jäntti, Camilla

    2016-06-01

    It is unclear whether mortality still is increased in acromegaly and whether there are gender-related differences. We dynamically assessed outcome during long-term follow-up in our nationwide cohort. We studied standardized mortality ratios (SMRs) relative to the general population and causes of death in acromegaly (n=333) compared with age- and gender-matched controls (n=4995). During 20 (0-33) years follow-up, 113 (34%) patients (n=333, 52% women) and 1334 (27%) controls (n=4995) died (P=0.004). SMR (1.9, 95% CI: 1.53-2.34, Pacromegaly. Overall distribution of causes of death (Pacromegaly, but not in controls, causes of deaths shifted from 44% cardiovascular and 28% cancer deaths during the first decade, to 23% cardiovascular and 35% cancer deaths during the next two decades. In acromegaly, cancer deaths were mostly attributed to pancreatic adenocarcinoma (n=5), breast (n=4), lung (n=3) and colon (n=3) carcinoma. In acromegaly, men were younger than women at diagnosis (median 44.5 vs 50 years, Pacromegaly, men are younger at diagnosis and death than women. Compared with controls, mortality is increased during 20 years of follow-up, especially in women. Causes of deaths shift from predominantly cardiovascular to cancer deaths. © 2016 Society for Endocrinology.

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

    NARCIS (Netherlands)

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

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

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

    Science.gov (United States)

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

    2016-06-01

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

  11. Neurocognitive function in acromegaly after surgical resection of GH-secreting adenoma versus naïve acromegaly.

    Directory of Open Access Journals (Sweden)

    Juan Francisco Martín-Rodríguez

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

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

    Science.gov (United States)

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

    2013-01-01

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

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

    Science.gov (United States)

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

    2014-01-01

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

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

    Directory of Open Access Journals (Sweden)

    S. Caprio

    2011-09-01

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

  15. Predictors of endoscopic transsphenoidal surgery outcome in acromegaly: patient and tumor characteristics evaluated by magnetic resonance imaging

    NARCIS (Netherlands)

    van Bunderen, Christa C.; van Varsseveld, Nadège C.; Baayen, Johannes C.; van Furth, Wouter R.; Aliaga, Esther Sanchez; Hazewinkel, Marieke J.; Majoie, Charles B. L. M.; Freling, Nicole J. M.; Lips, Paul; Fliers, Eric; Bisschop, Peter H.; Drent, Madeleine L.

    2013-01-01

    The availability of various first-line treatment modalities for acromegaly and evolving surgical techniques emphasize the need for accurately defined predictors of surgical outcome. We retrospectively analysed the outcome of 30 patients with acromegaly after initial endoscopic transsphenoidal

  16. Pasireotide: a novel treatment for patients with acromegaly

    Directory of Open Access Journals (Sweden)

    Cuevas-Ramos D

    2016-01-01

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

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

    Science.gov (United States)

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

    2016-05-01

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

  18. Comparative analysis of brain X-ray computed tomography in patients with acromegaly before and after gamma-ray teletherapy

    International Nuclear Information System (INIS)

    Balkanov, A.S.; Stashuk, G.A.; Polyakov, P.Yu.; Sherman, L.A.; Bychenkov, O.A.

    1999-01-01

    Results are analysed of the application of X-ray computerized tomography (KT) of brain in acromegaly patients before and after remote gamma therapy (RGT). Efficiency is shown of the KT method permitting both to reveal pituitary adenomas in case of acromegaly and to be applicable in the combination with other methods to assessment of the RGT efficiency when treating acromegaly [ru

  19. Acromegaly associated with a symptomatic Rathke′s cyst

    Directory of Open Access Journals (Sweden)

    Vishal Gupta

    2011-01-01

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

  20. Body composition in acromegaly - before and after therapy

    International Nuclear Information System (INIS)

    Brummer, R.J.M.; Bengtsson, B.A.; Isaksson, B.

    1986-01-01

    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 42 K by dilution technique or by counting gamma radiation from the naturally present 40 K 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

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

    International Nuclear Information System (INIS)

    Rosenstock, J.; Doyle, F.H.; Joplin, G.F.; Jung, R.T.; Mashiter, K.

    1982-01-01

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

  2. Acromegaly Quality of Life Questionnaire (AcroQoL

    Directory of Open Access Journals (Sweden)

    Lara Nuria

    2004-02-01

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

  3. Persistence of Diabetes and Hypertension After Multimodal Treatment of Acromegaly.

    Science.gov (United States)

    González, Baldomero; Vargas, Guadalupe; de Los Monteros, Ana Laura Espinosa; Mendoza, Victoria; Mercado, Moisés

    2018-06-01

    Diabetes and hypertension are frequent comorbidities of acromegaly. To analyze the course of diabetes and hypertension at diagnosis and after multimodal therapy in a large cohort of patients with acromegaly. Retrospective study at a tertiary care center. A total of 522 patients with acromegaly treated according to a preestablished protocol. Prevalence of diabetes and hypertension and its relationship with biochemical indices of acromegalic control. The cohort was stratified according to disease activity upon last visit to clinic: (1) surgical remission (n = 122), (2) pharmacologically controlled (n = 92), (3) active disease (n = 148), (4) insulinlike growth factor (IGF)-1 discordance (n = 64), and (5) growth hormone (GH) discordance (n = 96). The prevalence of diabetes and hypertension at diagnosis was 30% and 37%, respectively, and did not change upon the last visit (30.6% and 38%). Both comorbidities were more prevalent at diagnosis and on the last visit than in the general population. Diabetes was less prevalent on the last visit in patients who achieved surgical remission than in those who persisted with active disease (25% vs 40%, P = 0.01). Upon multivariate analysis, diabetes was associated with an IGF-1 at diagnosis >2× upper limit of normal, with the persistence of active acromegaly, the presence of hypertension upon the last visit, with the presence of a macroadenoma, and with female sex. Our findings underscore the importance of an integral approach when managing these patients, focusing not only on the control of GH and IGF-1 levels but also on the timely diagnosis and the specific treatment of each comorbidity.

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

    Science.gov (United States)

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

    2017-06-01

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

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

    Science.gov (United States)

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

    2016-10-01

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

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

    Energy Technology Data Exchange (ETDEWEB)

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

    1982-03-01

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

  7. Locking of metacarpophalangeal joints in a patient with acromegaly

    Energy Technology Data Exchange (ETDEWEB)

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

    1999-11-01

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

  8. Locking of metacarpophalangeal joints in a patient with acromegaly

    International Nuclear Information System (INIS)

    Tani, Y.; Tanaka, N.; Isoya, Eiji

    1999-01-01

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

  9. The role of primary radiotherapy in acromegaly (abstract)

    International Nuclear Information System (INIS)

    Ali, M.; Knob, G.

    1998-01-01

    The aim of the study was to evaluate the role of primary radiotherapy in acromegaly. Fifteen cases were randomly included in this study from 1989 to 1998. The patients were given external radiotherapy using Co/sup 60/ source and 200 cGy/F, 5F/week up to 5000 cGy, Tumor dose (TD). The target volume was irradiated by opposite parallel lateral field. The field size ranged from 4.5 to 6.5 cm. depending upon the tumor size and its extensions. These patients were continuously followed up for variable period depending upon the history of the patient and compliance by serial Growth Hormone (GH) assay and Clinical and Radiological evaluation. Out of these fifteen patients one died of cardiac sequelae. In the remaining treated patients majority has normal or continuously decreasing GH levels. The treatment of Acromegaly includes surgery, radiotherapy, medical treatment or the combination of these with variable success and non is perfect. We used radiotherapy as the primary modality, due to medical contraindication or refusal of patient for surgery and found that this is an effective modality in the treatment of acromegaly even in patients with extra sellar extension of the tumor. (author)

  10. The exon-3 deleted growth hormone receptor polymorphism predisposes to long-term complications of acromegaly

    NARCIS (Netherlands)

    Wassenaar, M. J. E.; Biermasz, N. R.; Pereira, A. M.; van der Klaauw, A. A.; Smit, J. W. A.; Roelfsema, F.; van der Straaten, T.; Cazemier, M.; Hommes, D. W.; Kroon, H. M.; Kloppenburg, M.; Guchelaar, H.-J.; Romijn, J. A.

    2009-01-01

    The aim of the study was to evaluate the impact of the genomic deletion of exon 3 of the GH receptor (d3GHR) on long-term clinical outcome of acromegaly in a well-characterized cohort of patients with long-term remission of acromegaly. We conducted a cross-sectional study. The presence of the d3GHR

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

    Science.gov (United States)

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

    2015-01-01

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

  12. Acromegaly is associated with an increased prevalence of colonic diverticula: a case-control study

    NARCIS (Netherlands)

    Wassenaar, M. J. E.; Cazemier, M.; Biermasz, N. R.; Pereira, A. M.; Roelfsema, F.; Smit, J. W. A.; Hommes, D. W.; Felt-Bersma, R. J. F.; Romijn, J. A.

    2010-01-01

    In acromegaly, overproduction of GH and IGF-I causes abnormal extracellular matrix regulation. We hypothesized that this may predispose to the development of colonic diverticula. Because the relation between acromegaly and colonic diverticula is unknown, the study aim was to assess the prevalence of

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

    NARCIS (Netherlands)

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

    2009-01-01

    The introduction of effective pharmacological treatments has changed the management of acromegaly. However, chronic, life-long treatment with somatostatin analogues and/or growth hormone receptor antagonists is very expensive. We estimated the costs of treatment algorithms to control acromegaly from

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

    NARCIS (Netherlands)

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

    2012-01-01

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

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

    Science.gov (United States)

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

    2010-10-01

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

  16. Trabecular bone score as a skeletal fragility index in acromegaly patients.

    Science.gov (United States)

    Hong, A R; Kim, J H; Kim, S W; Kim, S Y; Shin, C S

    2016-03-01

    Lumbar spine trabecular bone score (TBS) was significantly decreased in active acromegaly patients. TBS may be useful to assess the skeletal fragility in acromegaly in which bone mineral density (BMD) is not sufficient to represent bone strength and explain the high incidence of fragility fractures in acromegaly patients. Although the data on BMD are controversial, patients with acromegaly have an increased risk of fragility fracture. We examined the lumbar spine TBS to explain the skeletal deterioration in acromegaly patients. We included 14 men and 19 women acromegaly patients who underwent dual-energy X-ray absorptiometry at the time of diagnosis from 2000 to 2014 at Seoul National University Hospital. Ninety-nine age-, sex- and body mass index-matched controls were recruited. Biochemical parameters, lumbar spine TBS, and BMD at all sites were measured. Gonadal status was evaluated at diagnosis. Lumbar spine TBS was lower in acromegaly patients than in controls in both genders (1.345 ± 0.121 vs. 1.427 ± 0.087, P = 0.005 in men; 1.356 ± 0.082 vs. 1.431 ± 0.071, P = 0.001 in women). In contrast, BMD at all sites did not differ between the two groups. Hypogonadal acromegaly patients (men, n = 9; women, n = 12) had lower TBS values compared with controls both in men and women (all P acromegaly patients, lumbar spine TBS was lower than in women controls only (P = 0.041). Skeletal microarchitecture was deteriorated in acromegaly patients as assessed by TBS, which seems to be a consequence of growth hormone excess as well as hypogonadism, especially in women.

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

    Science.gov (United States)

    Christofides, Elena A

    2016-01-01

    In acromegaly, achieving biochemical control (growth hormone [GH] level acromegaly is challenging because it is rooted in observing subtle clinical manifestations, and it is typical for acromegaly to evolve for up to 10 years before it is recognized. This results in chronic exposure to elevated levels of GH and IGF-1 and delay in patients receiving appropriate treatment, which consequently increases mortality risk. In this review, the clinical impact of elevated GH and IGF-1 levels, the effectiveness of current therapies, and the potential role of novel treatments for acromegaly will be discussed. Clinical burden of acromegaly and benefits associated with management of GH and IGF-1 levels will be reviewed. Major treatment paradigms in acromegaly include surgery, medical therapy, and radiotherapy. With medical therapies, such as somatostatin analogs, dopamine agonists, and GH receptor antagonists, a substantial proportion of patients achieve reduced GH and normalized IGF-1 levels. In addition, signs and symptoms, quality of life, and comorbidities have also been reported to improve to varying degrees in patients who achieve biochemical control. Currently, there are several innovative therapies in development to improve patient outcomes, patient use, and access. Timely biochemical control of acromegaly ensures that the patient can ultimately improve morbidity and mortality from this disease and its extensive consequences. PMID:27471378

  18. Bone microarchitecture and estimated bone strength in men with active acromegaly.

    Science.gov (United States)

    Silva, Paula P B; Amlashi, Fatemeh G; Yu, Elaine W; Pulaski-Liebert, Karen J; Gerweck, Anu V; Fazeli, Pouneh K; Lawson, Elizabeth; Nachtigall, Lisa B; Biller, Beverly M K; Miller, Karen K; Klibanski, Anne; Bouxsein, Mary; Tritos, Nicholas A

    2017-11-01

    Both acromegaly and adult growth hormone deficiency (GHD) are associated with increased fracture risk. Sufficient data are lacking regarding cortical bone microarchitecture and bone strength, as assessed by microfinite element analysis (µFEA). To elucidate both cortical and trabecular bone microarchitecture and estimated bone strength in men with active acromegaly or GHD compared to healthy controls. Cross-sectional study at a clinical research center, including 48 men (16 with acromegaly, 16 with GHD and 16 healthy controls). Areal bone mineral density (aBMD), cortical and trabecular bone microarchitecture and estimated bone strength (µFEA) at the radius and tibia. aBMD was not different between the 3 groups at any skeletal site. At the radius, patients with acromegaly had greater cortical area ( P  acromegaly had lower trabecular bone density ( P  = 0.0082), but no differences in cortical bone microstructure. Compressive strength and failure load did not significantly differ between groups. These findings persisted after excluding patients with hypogonadism. Bone microarchitecture was not deficient in patients with GHD. Both cortical and trabecular microarchitecture are altered in men with acromegaly. Our data indicate that GH excess is associated with distinct effects in cortical vs trabecular bone compartments. Our observations also affirm the limitations of aBMD testing in the evaluation of patients with acromegaly. © 2017 European Society of Endocrinology.

  19. Acromegaly Presenting With Bilateral Vocal Fold Immobility: Case Report and Review of the Literature.

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    Cooper, Timothy; Dziegielewski, Peter T; Singh, Praby; Seemann, Robert

    2016-11-01

    To present a case of bilateral vocal fold immobility (BVCI) in a patient with acromegaly and review the current literature describing this presentation. Case report and literature review. Academic tertiary care center. English language literature search of online journal databases. A 56-year-old man presented with 3 months of progressive stridor and shortness of breath. Transnasal flexible endoscopy revealed BVCI. A tracheostomy was performed to secure his airway. Further history was suggestive of acromegaly and imaging demonstrated a pituitary macroadenoma. The diagnosis of acromegaly was made. The patient was treated with octreotide followed by an endoscopic trans sphenoidal resection of the pituitary adenoma. Sixteen months after his initial presentation, a right laser arytenoidectomy was performed and the patient was subsequently decannulated. In the literature to date, 11 cases of BVCI in acromegaly have been reported. These patients often present with stridor and require a tracheostomy. With treatment of their acromegaly, these patients may regain vocal fold mobility and may be decannulated. Acromegaly with BVCI is a rare presentation. Acute management of the airway of patients with acromegaly presenting with BVCI typically requires a tracheostomy. A period of 15 months should be allowed for restoration of vocal fold mobility before airway opening procedures are considered. Copyright © 2016 The Voice Foundation. Published by Elsevier Inc. All rights reserved.

  20. [Acromegaly and pregnancy: report of six new cases].

    Science.gov (United States)

    Persechini, M-L; Gennero, I; Grunenwald, S; Vezzosi, D; Bennet, A; Caron, P

    2014-11-01

    Pregnancies in acromegalic women are rare. Data from the literature indicate absence of congenital malformation in newborns, an increase of pituitary adenoma volume rarely clinically symptomatic, an increased risk of gestational diabetes and gravid hypertension in women with non-controlled GH/IGF-1 hypersecretion before gestation. The changes of somatotroph function are rarely described. Report of six new pregnancies in five women with acromegaly. Before pregnancy three women had incomplete surgical resection of GH-secreting pituitary adenoma, all were treated with somatostatin analogues, and the medical treatment was withdrawal at the diagnosis of gestation. We studied clinical (blood pressure, headaches, visual field), biological (blood glucose concentration) signs, GH and IGF-1 levels were measured during each trimester of pregnancy as well as in post-partum and were compared with pregestational values, MRI of the pituitary performed during the second trimester and in the post-partum were compared with MRI examen before pregnancy. All those pregnancies were normal without gestational diabetes, gravid hypertension and pituitary tumor syndrome. Clinical signs of acromegaly improved in 50 % of the patients, and IGF-1 decreased (22 %) in comparison of pregestational value without significant change in GH levels. No newborn had congenital malformation. Pregnancies in those women with acromegaly are uneventful without obstetrical or foetal complication, but a maternal follow-up is necessary in order to diagnose gravid hypertension and gestational diabetes. On the other hand, a clinical monitoring of pituitary tumor syndrome is necessary in women with non-operated GH-secreting macroadenoma before pregnancy. During the first trimester of gestation, an improvement of acromegalic signs can be due to a decrease of IGF-1 levels related to hepatic GH-resistance state secondary to physiological secretion of estrogens during gestation. Copyright © 2013 Elsevier Masson

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

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

    2016-07-01

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

  2. Body Composition and Ectopic Lipid Changes With Biochemical Control of Acromegaly.

    Science.gov (United States)

    Bredella, Miriam A; Schorr, Melanie; Dichtel, Laura E; Gerweck, Anu V; Young, Brian J; Woodmansee, Whitney W; Swearingen, Brooke; Miller, Karen K

    2017-11-01

    Acromegaly is characterized by growth hormone (GH) and insulinlike growth factor-1 (IGF-1) hypersecretion, and GH and IGF-1 play important roles in regulating body composition and glucose homeostasis. The purpose of our study was to investigate body composition including ectopic lipids, measures of glucose homeostasis, and gonadal steroids in patients with active acromegaly compared with age-, body mass index (BMI)-, and sex-matched controls and to determine changes in these parameters after biochemical control of acromegaly. Cross-sectional study of 20 patients with active acromegaly and 20 healthy matched controls. Prospective study of 16 patients before and after biochemical control of acromegaly. Body composition including ectopic lipids by magnetic resonance imaging/proton magnetic resonance spectroscopy; measures of glucose homeostasis by an oral glucose tolerance test; gonadal steroids. Patients with active acromegaly had lower mean intrahepatic lipid (IHL) and higher mean fasting insulin and insulin area under the curve (AUC) values than controls. Men with acromegaly had lower mean total testosterone, sex hormone-binding globulin, and estradiol values than male controls. After therapy, homeostasis model assessment of insulin resistance, fasting insulin level, and insulin AUC decreased despite an increase in IHL and abdominal and thigh adipose tissues and a decrease in muscle mass. Patients with acromegaly were characterized by insulin resistance and hyperinsulinemia but lower IHL compared with age-, BMI-, and sex-matched healthy controls. Biochemical control of acromegaly improved insulin resistance but led to a less favorable anthropometric phenotype with increased IHL and abdominal adiposity and decreased muscle mass. Copyright © 2017 Endocrine Society

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

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    Najjari, Mohsen

    2015-10-01

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

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

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    Arya Kavita

    1997-01-01

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

  5. Transsphenoidal microsurgery in the treatment of acromegaly and gigantism.

    Science.gov (United States)

    Arafah, B U; Brodkey, J S; Kaufman, B; Velasco, M; Manni, A; Pearson, O H

    1980-03-01

    Twenty-five patients with acromegaly and 3 patients with gigantism underwent transsphenoidal microsurgery in an attempt to remove the tumor and preserve normal pituitary function whenever possible. An adenoma was identified and removed in 27 of 28 patients. Evaluation 3--6 months postoperatively revealed a GH level less than 5 ng/ml in 29 patients, 5--10 ng/ml in 4 patients and 11--29 ng/ml in 4 other patients. Dynamics of GH secretion were normal in 11 patients who had normal pituitary function and are considered cured. Two patients with low or undetectable GH levels are also considered cured at the expense of being hypopituitary. Three of 7 patients with normal basal GH levels but abnormal dynamics of GH secretion relapsed within 1 yr. Eleven of the 13 patients considered cured did not have extrasellar extension, while 14 of the 15 patients not cured had extrasellar extension. Five patients who were not cured with surgery received radiation therapy. Three patients were treated with an ergot derivative, Lergotrile mesylate, after surgery and radiation therapy failed to normalize GH levels. Transsphenoidal microsurgery is an optimal form of therapy for patients with acromegaly or gigantism, especially those with no extrasellar extension. Dynamics of GH secretion are very useful in evaluating the completeness of adenoma removal.

  6. Transnasal stereotactic surgery of pituitary adenomas concomitant with acromegaly.

    Science.gov (United States)

    Metyolkina, L; Peresedov, V

    1995-01-01

    Since 1960 we have performed stereotactic transsphenoidal cryohypophysectomy in 70 patients with pituitary adenomas, 42 women and 28 men, aged 11-59 years. The dominant clinical syndrome was acromegaly in 50 patients, galactorrhea in 9, amenorrhea in 5, adiposogenital dystrophy in 4 and gigantism with mild endocrine symptomatology in 2 patients. In 67 patients the histological structure of the tumor was established by biopsy (50 patients with eosinophil adenoma, 10 with mixed-type adenoma, 4 with chromophobe adenoma and 3 with basophil adenoma). Somatotropic hormone, human growth hormone, prolactin, ACTH and 17-ketosteroid levels indicated active/inactive adenomas. In 42 cases the adenoma was only intrasellar, which was confirmed by contrast X-ray investigations, CT scanning, angiography and ophthalmological investigation. Transnasal stereotactic cryohypophysectomy was performed in all 70 cases using a stereotactic apparatus especially designed for operations on the pituitary. All patients (except 2) tolerated the operation well. No complications occurred. Vision deteriorated after operation in 1 patient. Thrombosis of the left middle cerebral artery developed in another patient. All the other patients noted improvement directly after operation - rapid diminution of signs of acromegaly and rapid restoration of normal values in hormonal tests. Six patients with continuing growth of the tumor underwent a second operation 1.5-6 years after the first operation. We conclude from our own clinical experience and information from the literature that transnasal stereotactic cryodestruction is highly effective and relatively safe in the management of pituitary adenoma.

  7. Risk factors for glucose intolerance in active acromegaly

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    Kreze A.

    2001-01-01

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

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

    Science.gov (United States)

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

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

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

    Science.gov (United States)

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

    2017-03-01

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

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

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    V N Azizyan

    2011-03-01

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

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

    Science.gov (United States)

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

    2015-01-01

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

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

    Science.gov (United States)

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

    2016-08-01

    Acromegaly is a rare disease with a large burden due its associated comorbidities and the life-long management required. Since the occurrence and severity of associated complications are related to length of exposure to the excess growth hormone seen in acromegaly, early diagnosis is imperative. The delay in diagnosis, however, can be long, and may be the result of a lack of disease awareness and screening programs. Since acromegaly is an uncommon disease, finding ways to increase recognition and diagnosis that would permit early detection in a logical and cost-effective manner could be a challenge. We conducted a retrospective literature review for information relating to the screening and diagnosis of acromegaly using PubMed. The aim was to assess whether an acromegaly-screening program in Latin America (and elsewhere) would be both of use and be feasible. An earlier diagnosis allows earlier initiation of treatment, such as surgery and/or drugs, which leads to more successful disease management (biochemical control) and better outcomes. Since the delay in diagnosis can be long, we believe that clear opportunities exist for earlier (and increased) detection of acromegaly. This can be achieved by increasing disease awareness for earlier recognition of symptoms and by using targeted screening (rather than mass screening) programs.

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

    Science.gov (United States)

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

    2017-01-01

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

  14. Pegvisomant: a growth hormone receptor antagonist used in the treatment of acromegaly.

    Science.gov (United States)

    Tritos, Nicholas A; Biller, Beverly M K

    2017-02-01

    To review published data on pegvisomant and its therapeutic role in acromegaly. Electronic searches of the published literature were conducted using the keywords: acromegaly, growth hormone (GH) receptor (antagonist), pegvisomant, therapy. Relevant articles (n = 141) were retrieved and considered for inclusion in this manuscript. Pegvisomant is a genetically engineered, recombinant growth hormone receptor antagonist, which is effective in normalizing serum insulin-like growth factor 1 (IGF-1) levels in the majority of patients with acromegaly and ameliorating symptoms and signs associated with GH excess. Pegvisomant does not have direct antiproliferative effects on the underlying somatotroph pituitary adenoma, which is the etiology of GH excess in the vast majority of patients with acromegaly. Therefore, patients receiving pegvisomant monotherapy require regular pituitary imaging in order to monitor for possible increase in tumor size. Adverse events in patients on pegvisomant therapy include skin rashes, lipohypertrophy at injection sites, and idiosyncratic liver toxicity (generally asymptomatic transaminitis that is reversible upon drug discontinuation), thus necessitating regular patient monitoring. Pegvisomant is an effective therapeutic agent in patients with acromegaly who are not in remission after undergoing pituitary surgery. It mitigates excess GH action, as demonstrated by IGF-1 normalization, but has no direct effects on pituitary tumors causing acromegaly. Regular surveillance for possible tumor growth and adverse effects (hepatotoxicity, skin manifestations) is warranted.

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

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    Ali Riza Koksal

    2014-01-01

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

  16. Relationship of apelin, procalcitonin, and fetuin-A concentrations with carotid intima-media thickness in acromegaly.

    Science.gov (United States)

    Topsakal, S; Akin, F; Turgut, S; Yaylali, G F; Herek, D; Ayada, C

    2015-07-01

    Acromegaly is characterized by excess growth hormone and insulin-like growth factor-1 concentrations. There is conflicting evidence as to whether acromegaly is associated with an increased risk of atherosclerosis. Apelin is an adipose tissue-derived peptide that may be associated with hyperinsulinemia. Fetuin-A is a hepatocyte produced plasma glycoprotein that has an important role as a calcification inhibitor. The aim of this study was to examine apelin, fetuin-A, and procalcitonin concentrations and to assess their relationship with carotid intima medial thickness (cIMT) in subjects with acromegaly. Apelin, fetuin-A, and procalcitonin serum concentrations were measured in 37 (20 inactive and 17 active) subjects with acromegaly and 30 control subjects, along with carotid intima medial thickness. The concentrations of apelin, fetuin-A, and procalcitonin were increased in subjects with acromegaly. There were significant correlations between apelin, fetuin-A, and procalcitonin in subjects with acromegaly. Carotid intima medial thickness values were similar between control subjects and subjects with acromegaly. Carotid intima medial thickness was not increased in subjects with acromegaly. It is possible that the increased apelin and fetuin-A concentrations observed play a protective role against the development of atherosclerosis in subjects with acromegaly. © The Author(s) 2015 Reprints and permissions: sagepub.co.uk/journalsPermissions.nav.

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

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    Ming-Ying Lu

    2006-06-01

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

  18. The role of diabetes in acromegaly associated neoplasia.

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    Sonia Cheng

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

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

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    Kim, Hee Kyung; Lee, Ji Shin; Park, Min Ho; Cho, Jin Seong; Yoon, Jee Hee; Kim, Soo Jeong; Kang, Ho-Cheol

    2014-01-01

    Introduction Several studies have reported a high frequency of papillary thyroid cancer (PTC) in patients with acromegaly. The aim of this study was to determine the prevalence and predictors of thyroid cancer in patients with acromegaly and to investigate the frequency of the BRAF V600E mutation in PTC patients with and without acromegaly. Materials and Methods We conducted a retrospective study of 60 patients with acromegaly. Thyroid ultrasonography (US) and US-guided fine needle aspiration were performed on nodules with sonographic features of malignancy. We selected 16 patients with non-acromegalic PTC as a control group. The BRAF V600E 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 BRAF V600E 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 development of

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

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    Hee Kyung Kim

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

  1. Acromegaly presented as a cause of laryngeal dyspnea.

    Science.gov (United States)

    Saussez, S; Mahillon, V; Chantrain, G; Thill, M P; Lequeux, T

    2007-12-01

    Acromegalic patients can develop obstructive sleep apnea syndrome or upper airflow obstruction. The development of dyspnea is unusual and the fixation of both vocal cords is exceptional. We report the case of a patient with bilateral vocal cord paralysis. Fiberoptic laryngoscopy and computed tomography (CT) of the neck showed a supra-glottic stenosis due to a swelling of the soft tissue. A tracheostomy was first performed. Thereafter, micro-laryngoscopy using laser vaporisation of the supra-glottic soft tissue was attempted but failed to remove the tracheostomy canula. Finally, blood tests and cerebral MRI revealed an acromegaly. The patient underwent a trans-sphenoidal resection of the pituitary adenoma. Fifteen months later, fiberoptic laryngoscopy showed bilateral restoration of vocal cord mobility and the tracheostomy canula was successfully removed after 18 months. Vocal cord fixation is probably due to hypopharyngeal and laryngeal soft tissue swelling and can be reversible after successful treatment of the adenoma.

  2. Long-term effects of megavoltage radiotherapy in acromegaly

    International Nuclear Information System (INIS)

    Lam, K.S.L.; Wang, C.; Ma, J.T.C.; Yeung, R.T.T.; Choi, P.

    1989-01-01

    The progress of 41 Chinese patients with acromegaly treated with megavoltage radiotherapy was reviewed after a mean follow-up of 4.5 (one-ten) years. Nine received prior surgery. Radiotherapy was delivered by a 3-field technique to a total of 4000-5000 cGy in 25 fractions. By life table analysis successful treatment of growth hormone (GH) hypersecretion, as defined by a mean GH concentration of ≤ 10 mu/L, could be expected in 6, 11, 26, 64 and 67% of the patients after one, two, five, eight and ten years respectively. Median intervals before achieving a mean GH level of ≤ 10 Mu/L were 6.6 and 8.6 years following radiotherapy with the higher (4500-5000 cGy) and lower (4000 cGy) doses respectively, suggesting a tendency towards earlier response following radiotherapy with the higher dose. The prevalence of acquired hypopituitarism in patients followed up for over five years was 40% for gonadotrophins, 30% for TSH and 20% for ACTH deficiency respectively. In the majority of patients, acquired hypopituitarism was not apparent within five years after radiotherapy. No mortality or major side effects were noted following radiotherapy. In 34 patients on longterm bromocriptine treatment, mean GH concentrations were normalised in 26.5% of patients. We conclude that with judicious selection of patients and use of adjunctive medical therapy, megavoltage radiotherapy remains a safe and satisfactory form of treatment for acromegaly especially if expert transphenoidal surgery is not readily available. 19 refs., 3 figs., 2 tabs

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

    Science.gov (United States)

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

    2015-01-01

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

  4. Two-dimensional speckle tracking echocardiography demonstrates no effect of active acromegaly on left ventricular strain.

    Science.gov (United States)

    Volschan, I C M; Kasuki, L; Silva, C M S; Alcantara, M L; Saraiva, R M; Xavier, S S; Gadelha, M R

    2017-06-01

    Speckle tracking echocardiography (STE) allows for the study of myocardial strain (ε), a marker of early and subclinical ventricular systolic dysfunction. Cardiac disease may be present in patients with acromegaly; however, STE has never been used to evaluate these patients. To evaluate left ventricular (LV) global longitudinal strain in patients with active acromegaly with normal LV systolic function. Cross-sectional clinical study. Patients with active acromegaly with no detectable heart disease and a control group were matched for age, gender, arterial hypertension and diabetes mellitus underwent STE. Global LV longitudinal ε (GLS), left ventricular mass index (LVMi), left ventricular ejection fraction (LVEF) and relative wall thickness (RWT) were obtained via two-dimensional (2D) echocardiography using STE. Thirty-seven patients with active acromegaly (mean age 45.6 ± 13.8; 48.6% were males) and 48 controls were included. The mean GLS was not significantly different between the acromegaly group and the control group (in %, -20.1 ± 3.1 vs. -19.4 ± 2.2, p = 0.256). Mean LVMi was increased in the acromegaly group (in g/m 2 , 101.6 ± 27.1 vs. 73.2 ± 18.6, p Acromegaly patients, despite presenting with a higher LVMi when analyzed by 2D echocardiography, did not present with impairment in the strain when compared to a control group; this finding indicates a low chance of evolution to systolic dysfunction and agrees with recent studies that show a lower frequency of cardiac disease in these patients.

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

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

    2017-05-01

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

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

    Science.gov (United States)

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

    2016-05-01

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

  7. Central ghrelin production does not substantially contribute to systemic ghrelin concentrations: a study in two subjects with active acromegaly

    NARCIS (Netherlands)

    F.M. van der Toorn (Fanny); W.W. de Herder (Wouter); F. Broglio (Fabio); E. Ghigo (Ezio); A-J. van der Lely (Aart-Jan); J.A.M.J.L. Janssen (Joseph)

    2002-01-01

    textabstractINTRODUCTION: In an animal model of acromegaly (PEPCK-hGH transgenic mice), low systemic levels of ghrelin have been observed compared with normal mice. We hypothesized that systemic circulating ghrelin levels are also decreased in humans with active acromegaly and

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

    LENUS (Irish Health Repository)

    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.

  9. Long-term maintenance of the anabolic effects of GH on the skeleton in successfully treated patients with acromegaly

    NARCIS (Netherlands)

    Biermasz, Nienke R.; Hamdy, Neveen A. T.; Pereira, Alberto M.; Romijn, Johannes A.; Roelfsema, Ferdinand

    2005-01-01

    The anabolic actions of growth hormone (GH) are well documented. In acromegaly, the skeletal effects of chronic GH excess have been mainly addressed by evaluating bone mineral density (BMD). Most data were obtained in patients with active acromegaly, and apparently high or normal BMD was observed in

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

    NARCIS (Netherlands)

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

    2011-01-01

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

  11. Increased psychopathology and maladaptive personality traits, but normal cognitive functioning, in patients after long-term cure of acromegaly

    NARCIS (Netherlands)

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

    2010-01-01

    Active acromegaly is associated with psychopathology, personality changes, and cognitive dysfunction. It is unknown whether, and to what extent, these effects are present after long-term cure of acromegaly. The aim of the study was to assess psychopathology, personality traits, and cognitive

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

    Science.gov (United States)

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

    2016-11-01

    Vertebral fractures are an emerging complication of acromegaly but their prediction is still difficult occurring even in patients with normal bone mineral density. In this study we evaluated the ability of high-resolution cone-beam computed tomography to provide information on skeletal abnormalities associated with vertebral fractures in acromegaly. 40 patients (24 females, 16 males; median age 57 years, range 25-72) and 21 healthy volunteers (10 females, 11 males; median age 60 years, range: 25-68) were evaluated for trabecular (bone volume/trabecular volume ratio, mean trabecular separation, and mean trabecular thickness) and cortical (thickness and porosity) parameters at distal radius using a high-resolution cone-beam computed tomography system. All acromegaly patients were evaluated for morphometric vertebral fractures and for mineral bone density by dual-energy X-ray absorptiometry at lumbar spine, total hip, femoral neck, and distal radius. Acromegaly patients with vertebral fractures (15 cases) had significantly (p acromegaly patients did not have significant differences in bone density at either skeletal site. Patients with acromegaly showed lower bone volume/trabecular volume ratio (p = 0.003) and mean trabecular thickness (p acromegaly. High-resolution cone-beam computed tomography at the distal radius may be useful to evaluate and predict the effects of acromegaly on bone microstructure.

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

    NARCIS (Netherlands)

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

    2007-01-01

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

  14. Matrix metalloproteinase 2 (MMP-2) levels are increased in active acromegaly patients.

    Science.gov (United States)

    Karci, Alper Cagri; Canturk, Zeynep; Tarkun, Ilhan; Cetinarslan, Berrin

    2017-07-01

    During follow-up of acromegaly patients, there is a discordance rate of 30% between the measurements of growth hormone and insulin-like growth factor-1 levels. Further tests are required to determine disease activity in patients with discordant results. This study was planned to investigate an association of serum levels of matrix metalloproteinase-2, matrix metalloproteinase-9, and cathepsin B with disease activity in acromegaly patients. In this study, 64 acromegaly patients followed in our clinic were divided into two groups according to the 2010 consensus criteria for cure of acromegaly as patients with active disease (n = 24) and patients with controlled disease (n = 40). Serum matrix metalloproteinase-2, matrix metalloproteinase-9, and cathepsin B levels were measured by the enzyme-linked immunosorbent assay method. The mean serum matrix metalloproteinase-2 level was significantly higher in the active acromegaly patients than in the controlled acromegaly patients (150.1 ± 54.5 ng/mL vs. 100.2 ± 44.6 ng/mL; p matrix metalloproteinase-9 and cathepsin B levels (p = 0.205 and p = 0.598, respectively). Serum matrix metalloproteinase-2 levels of 118.3 ng/mL and higher had a sensitivity of 75% and a specificity of 77.5% in determining active disease. The risk of active acromegaly was 3.3 fold higher in the patients with a matrix metalloproteinase-2 level of >118.3 ng/mL than in the patients with a matrix metalloproteinase-2 level of matrix metalloproteinase-2 level is increased in the active acromegaly patients and a threshold value in determining active disease was defined for serum matrix metalloproteinase-2 level. This study is the first to compare acromegaly patients having active or controlled disease in terms of matrix metalloproteinase-2 and matrix metalloproteinase-9 levels. The results need to be confirmed by a study that will be conducted in a larger patient group also including a healthy control group to demonstrate the

  15. Expression of microRNA related to bone remodeling regulation in plasma in patients with acromegaly

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    Tatiana A. Grebennikova

    2017-11-01

    Full Text Available Backgraund. MiсroRNA are small regulatory factors that regulate gene expression by post-transcriptional regulation of mRNA, playing an important role in numerous cellular processes including organogenesis, apoptosis, cell proliferation and differentiation. Acromegaly causes bone fragility, but the pathogenetic mechanism is generally unknown. Aim. To evaluate levels of microRNA related to bone remodeling regulation in plasma samples from patients with acromegaly Materials and methods. Fasting plasma samples were taken and stored in aliquot at ≤ -80°C from consecutive subjects with clinically evident and biochemically confirmed active acromegaly and healthy volunteers matched by age, sex and body mass index (BMI. miRNeasy Serum/Plasma Kit, TaqMan Advanced miRNA cDNA Synthesis Kit, TaqMan Advanced miRNA Assays were used to assay plasma miRNA expression. Insulin-like growth factor 1 (IGF1 was measured by immunochemiluminescence assay (Liaison. Results. We enrolled 40 subjects 22 patients suffered from acromegaly and 18 matched healthy controls matched by sex, age and BMI. The median age of patients with acromegaly was 42 years (Q25;Q75 – 37;43 with no difference among the groups, p=0.205; BMI – 28 (24;32 kg/m2, p=0.253. The median IGF1 in subjects with acromegaly – 622 (514;1000 ng/ml was significantly higher as compared to the control group (p<0.001. Patients with acromegaly had significantly higher expression of microRNA-100-5р (p=0.051, microRNA-550а-5р (p=0.048, microRNA-7b-5р (p=0.005 and microRNA-96-5р (p=0.042 among 27 bone-specific microRNA tested in plasma Conclusions. This study reveals that several microRNAs, known to regulate bone remodeling can be detected in plasma samples of patients with acromegaly and may be suggested as biomarkers for skeletal involvement in patients with acromegaly.

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

    Directory of Open Access Journals (Sweden)

    Hyae Min Lee

    2015-09-01

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

  17. Automatic Detection of Acromegaly From Facial Photographs Using Machine Learning Methods.

    Science.gov (United States)

    Kong, Xiangyi; Gong, Shun; Su, Lijuan; Howard, Newton; Kong, Yanguo

    2018-01-01

    Automatic early detection of acromegaly is theoretically possible from facial photographs, which can lessen the prevalence and increase the cure probability. In this study, several popular machine learning algorithms were used to train a retrospective development dataset consisting of 527 acromegaly patients and 596 normal subjects. We firstly used OpenCV to detect the face bounding rectangle box, and then cropped and resized it to the same pixel dimensions. From the detected faces, locations of facial landmarks which were the potential clinical indicators were extracted. Frontalization was then adopted to synthesize frontal facing views to improve the performance. Several popular machine learning methods including LM, KNN, SVM, RT, CNN, and EM were used to automatically identify acromegaly from the detected facial photographs, extracted facial landmarks, and synthesized frontal faces. The trained models were evaluated using a separate dataset, of which half were diagnosed as acromegaly by growth hormone suppression test. The best result of our proposed methods showed a PPV of 96%, a NPV of 95%, a sensitivity of 96% and a specificity of 96%. Artificial intelligence can automatically early detect acromegaly with a high sensitivity and specificity. Copyright © 2017 The Authors. Published by Elsevier B.V. All rights reserved.

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

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

    1990-01-01

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

  19. Renin-Angiotensin System Blockade Improves Cardiac Indices in Acromegaly Patients.

    Science.gov (United States)

    Thomas, Julia D J; Dattani, Abhishek; Zemrak, Filip; Burchell, Thomas; Akker, Scott A; Kaplan, Felicity J L; Khoo, Bernard; Aylwin, Simon; Grossman, Ashley B; Davies, L Ceri; Korbonits, Márta

    2017-06-01

    Blockade of the angiotensin-renin system, with angiotensin converting enzyme inhibitors (ACEi) and angiotensin receptor blockers (ARBs), has been shown to improve cardiac outcomes following myocardial infarction and delay progression of heart failure. Acromegaly is associated with a disease-specific cardiomyopathy, the pathogenesis of which is poorly understood.The cardiac indices of patients with active acromegaly with no hypertension (Group A, n=4), established hypertension not taking ACEi/ARBs (Group B, n=4) and established hypertension taking ACEi/ARBs (Group C, n=4) were compared using cardiac magnetic imaging.Patients taking ACEi/ARBs had lower end diastolic volume index (EDVi) and end systolic volume index (ESVi) than the other 2 groups ([C] 73.24 vs. [A] 97.92 vs. [B] 101.03 ml/m 2 , ANOVA p=0.034, B vs. C pAcromegaly patients on ACEi/ARBs for hypertension demonstrate improved cardiac indices compared to acromegaly patients with hypertension not taking these medications. Further studies are needed to determine if these drugs have a beneficial cardiac effect in acromegaly in the absence of demonstrable hypertension. © Georg Thieme Verlag KG Stuttgart · New York.

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

    Science.gov (United States)

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

    2016-06-27

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

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

    International Nuclear Information System (INIS)

    Soszynski, P.; Slowinska-Srzednicka, J.; Zgliczynski, S.

    1990-01-01

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

  2. Preoperative octreotide therapy and surgery in acromegaly: associations between glucose homeostasis and treatment response.

    Science.gov (United States)

    Helseth, R; Carlsen, S M; Bollerslev, J; Svartberg, J; Øksnes, M; Skeie, S; Fougner, S L

    2016-02-01

    In acromegaly, high GH/IGF-1 levels associate with abnormal glucose metabolism. Somatostatin analogs (SSAs) reduce GH and IGF-1 but inhibit insulin secretion. We studied glucose homeostasis in de novo patients with acromegaly and changes in glucose metabolism after treatment with SSA and surgery. In this post hoc analysis from a randomized controlled trial, 55 de novo patients with acromegaly, not using antidiabetic medication, were included. Before surgery, 26 patients received SSAs for 6 months. HbA1c, fasting glucose, and oral glucose tolerance test were performed at baseline, after SSA pretreatment and at 3 months postoperative. Area under curve of glucose (AUC-G) was calculated. Glucose homeostasis was compared to baseline levels of GH and IGF-1, change after SSA pretreatment, and remission both after SSA pretreatment and 3 months postoperative. In de novo patients, IGF-1/GH levels did not associate with baseline glucose parameters. After SSA pretreatment, changes in GH/IGF-1 correlated positively to change in HbA1c levels (both p acromegaly, disease activity did not correlate with glucose homeostasis. Surgical treatment of acromegaly improved glucose metabolism in both cured and not cured patients, while SSA pretreatment led to deterioration in glucose homeostasis in patients not achieving biochemical control.

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

    Directory of Open Access Journals (Sweden)

    Rajesh Jain

    2012-01-01

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

  4. Acromegaly-induced cardiomyopathy with dobutamine-induced outflow tract obstruction.

    Science.gov (United States)

    Abdelsalam, Mahmoud A; Nippoldt, Todd B; Geske, Jeffrey B

    2016-03-09

    A 50-year-old man with a history of acromegaly was referred for preoperative cardiac evaluation preceding trans-sphenoidal resection of a pituitary macroadenoma. Dobutamine stress echocardiography was negative for myocardial ischaemia. Resting left ventricular (LV) LV ejection fraction (LVEF) was 64% and there was hypertrophy of ventricular septum (18 mm) without resting LV outflow tract obstruction. With 40 µg/kg/min of dobutamine, the LVEF became hyperdynamic at 80%, and there was a maximal instantaneous LV outflow tract gradient of 77 mm Hg. There was no delayed myocardial enhancement on cardiac MRI and the pattern of hypertrophy was concentric. Acromegaly-induced cardiomyopathy can mimic hypertrophic cardiomyopathy in the setting of dobutamine provocation. Because cardiomyopathy is an important cause of mortality in acromegaly, diagnosis and appropriate management are critical to improve survival. 2016 BMJ Publishing Group Ltd.

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

    DEFF Research Database (Denmark)

    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......-stimulated (euglycemic clamp) substrate metabolism in seven patients with active acromegaly before and after 4-wk pegvisomant treatment (15 mg/d) in an open design. RESULTS: After pegvisomant, IGF-I decreased, whereas GH increased (IGF-I, 621 +/- 82 vs. 247 +/- 33 microg/liter, P = 0.02; GH, 5.3 +/- 1.5 vs. 10.8 +/- 3...... vs. 1563 +/- 101 kcal/24 h, P = 0.03), but the rate of lipid oxidation did not change significantly. CONCLUSIONS: 1) Pegvisomant treatment for 4 wk improves peripheral and hepatic insulin sensitivity in acromegaly. 2) This is associated with a decrease in resting energy expenditure, whereas free...

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

    DEFF Research Database (Denmark)

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

    2002-01-01

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

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

    Directory of Open Access Journals (Sweden)

    Deepak Khandelwal

    2011-01-01

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

  8. Stereotactic radiation therapy for the treatment of functional pituitary adenomas associated with feline acromegaly.

    Science.gov (United States)

    Wormhoudt, Tiffany L; Boss, Mary-Keara; Lunn, Katharine; Griffin, Lynn; Leary, Del; Dowers, Kristy; Rao, Sangeeta; LaRue, Susan M

    2018-05-21

    Conventional fractionated radiotherapy has been shown to be partially effective for management of pituitary tumors in cats that cause acromegaly and diabetes mellitus (DM), but, the efficacy and safety of stereotactic radiation therapy (SRT) as a treatment for acromegalic cats has not been described. Stereotactic radiation therapy is an effective and safe treatment for controlling acromegaly associated with pituitary adenomas in cats. Additionally, SRT-treated acromegalic cats with DM will experience a decrease in insulin requirements after radiation therapy. Fifty-three client-owned cats referred to Colorado State University for SRT to treat pituitary tumors causing poorly controlled DM secondary to acromegaly. Retrospective study of cats treated for acromegaly with SRT between 2008 and 2016 at Colorado State University. Diagnosis of acromegaly was based on history, physical examination, laboratory results, and cross-sectional imaging of the pituitary. Signalment, radiation protocol, insulin requirements over time, adverse effects, and survival were recorded. Median survival time was 1072 days. Of the 41 cats for which insulin dosage information was available, 95% (39/41) experienced a decrease in required insulin dose, with 32% (13/41) achieving diabetic remission. Remission was permanent in 62% (8/13) and temporary in 38% (5/13) cats. Median duration to lowest insulin dose was 9.5 months. Of the treated cats, 14% developed hypothyroidism and required supplementation after SRT. Stereotactic radiation therapy is safe and effective for treating cats with acromegaly. Cats treated with SRT have improved survival time and control of their DM when compared to previously reported patients treated with non-SRT. Copyright © 2018 The Authors. Journal of Veterinary Internal Medicine published by Wiley Periodicals, Inc. on behalf of the American College of Veterinary Internal Medicine.

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

    Science.gov (United States)

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

    2015-01-01

    Objective Patients with acromegaly exhibit reduced life expectancy and increased prevalence of age-related diseases, such as diabetes, hypertension, and cardiovascular disease. However, the underlying mechanism has not been fully elucidated. Telomere shortening is reportedly associated with reduced life expectancy and increased prevalence of these age-related diseases. Methods We measured telomere length in patients with acromegaly using quantitative PCR method. The effect of GH and IGF-I on telomere length and cellular senescence was examined in human skin fibroblasts. Results Patients with acromegaly exhibited shorter telomere length than age-, sex-, smoking-, and diabetes-matched control patients with non-functioning pituitary adenoma (0.62 ± 0.23 vs. 0.75 ± 0.35, respectively, P = 0.047). In addition, telomere length in acromegaly was negatively correlated with the disease duration (R 2 = 0.210, P = 0.003). In vitro analysis revealed that not GH but IGF-I induced telomere shortening in human skin fibroblasts. Furthermore, IGF-I-treated cells showed increased senescence-associated β-galactosidase activity and expression of p53 and p21 protein. IGF-I-treated cells reached the Hayflick limit earlier than GH- or vehicle-treated cells, indicating that IGF-I induces cellular senescence. Conclusion Shortened telomeres in acromegaly and cellular senescence induced by IGF-I can explain, in part, the underlying mechanisms by which acromegaly exhibits an increased morbidity and mortality in association with the excess secretion of IGF-I. PMID:26448623

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

    Directory of Open Access Journals (Sweden)

    Ryusaku Matsumoto

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

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

    Science.gov (United States)

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

    2016-08-01

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

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

    Science.gov (United States)

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

    2016-11-01

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

  13. Acromegaly incidence, prevalence, complications and long-term prognosis: a nationwide cohort study.

    Science.gov (United States)

    Dal, Jakob; Feldt-Rasmussen, Ulla; Andersen, Marianne; Kristensen, Lars Ø; Laurberg, Peter; Pedersen, Lars; Dekkers, Olaf M; Sørensen, Henrik Toft; Jørgensen, Jens Otto L

    2016-09-01

    Valid data on acromegaly incidence, complications and mortality are scarce. The Danish Health Care System enables nationwide studies with complete follow-up and linkage among health-related databases to assess acromegaly incidence, prevalence, complications and mortality in a population-based cohort study. All incident cases of acromegaly in Denmark (1991-2010) were identified from health registries and validated by chart review. We estimated the annual incidence rate of acromegaly per 10(6) person-years (py) with 95% confidence intervals (95% CIs). For every patient, 10 persons were sampled from the general population as a comparison cohort. Cox regression and hazard ratios (HRs) with 95% confidence intervals (95% CIs) were used. Mean age at diagnosis (48.7 years (CI: 95%: 47.2-50.1)) and annual incidence rate (3.8 cases/10(6) persons (95% CI: 3.6-4.1)) among the 405 cases remained stable. The prevalence in 2010 was 85 cases/10(6) persons. The patients were at increased risk of diabetes mellitus (HR: 4.0 (95% CI: 2.7-5.8)), heart failure (HR: 2.5 (95% CI: 1.4-4.5)), venous thromboembolism (HR: 2.3 (95% CI: 1.1-5.0)), sleep apnoea (HR: 11.7 (95% CI: 7.0-19.4)) and arthropathy (HR: 2.1 (95% CI: 1.6-2.6)). The complication risk was also increased before the diagnosis of acromegaly. Overall mortality risk was elevated (HR: 1.3 (95% CI: 1.0-1.7)) but uninfluenced by treatment modality. (i) The incidence rate and age at diagnosis of acromegaly have been stable over decades, and the prevalence is higher than previously reported. (ii) The risk of complications is very high even before the diagnosis. (iii) Mortality risk remains elevated but uninfluenced by mode of treatment. © 2016 European Society of Endocrinology.

  14. [The para-clinic investigation of temporo-mandibular joint changes in patients with acromegaly].

    Science.gov (United States)

    Morăraşu, C; Burlui, V; Olaru, C; Boza, C; Bortă, C; Morăraşu, G; Brînză, M

    2001-01-01

    The Acromegaly is an endocrinological disease determined by the hypersecretion of STH in a certain period of the body evolution and it causes the hypertrophy of bones in general and of mandible and cranio-facial bones, determining a disorder due to this development of bones, associated with troubles in the activity of muscles and of the phospho-calcium metabolism. This study was made on a group of 33 acromegaly patients. Their temporo-mandibular joint was investigated by ortopantomography, tomography, computer tomography and scintigraphy. All of these exams shows the changes in temporo-mandibular joint due to the cells hyperactivity determined by the hypersecretion of STH.

  15. Acromegaly determination using discriminant analysis of the three-dimensional facial classification in Taiwanese.

    Science.gov (United States)

    Wang, Ming-Hsu; Lin, Jen-Der; Chang, Chen-Nen; Chiou, Wen-Ko

    2017-08-01

    The aim of this study was to assess the size, angles and positional characteristics of facial anthropometry between "acromegalic" patients and control subjects. We also identify possible facial soft tissue measurements for generating discriminant functions toward acromegaly determination in males and females for acromegaly early self-awareness. This is a cross-sectional study. Subjects participating in this study included 70 patients diagnosed with acromegaly (35 females and 35 males) and 140 gender-matched control individuals. Three-dimensional facial images were collected via a camera system. Thirteen landmarks were selected. Eleven measurements from the three categories were selected and applied, including five frontal widths, three lateral depths and three lateral angular measurements. Descriptive analyses were conducted using means and standard deviations for each measurement. Univariate and multivariate discriminant function analyses were applied in order to calculate the accuracy of acromegaly detection. Patients with acromegaly exhibit soft-tissue facial enlargement and hypertrophy. Frontal widths as well as lateral depth and angle of facial changes were evident. The average accuracies of all functions for female patient detection ranged from 80.0-91.40%. The average accuracies of all functions for male patient detection were from 81.0-94.30%. The greatest anomaly observed was evidenced in the lateral angles, with greater enlargement of "nasofrontal" angles for females and greater "mentolabial" angles for males. Additionally, shapes of the lateral angles showed changes. The majority of the facial measurements proved dynamic for acromegaly patients; however, it is problematic to detect the disease with progressive body anthropometric changes. The discriminant functions of detection developed in this study could help patients, their families, medical practitioners and others to identify and track progressive facial change patterns before the possible patients

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

    Science.gov (United States)

    Silverstein, Julie M

    2016-10-01

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

  17. Targeting either GH or IGF-I during somatostatin analogue treatment in patients with acromegaly

    DEFF Research Database (Denmark)

    Dal, Jakob; Klose, Marianne; Heck, Ansgar

    2018-01-01

    CONTEXT: Discordant GH and IGF-I values are frequent in acromegaly. The clinical significance and its dependence on treatment modality and of glucose-suppressed GH (GHnadir) measurements remain uncertain. OBJECTIVE: To evaluate the effects of targeting either IGF-I or GH during somatostatin analog...... (SA) treatment. PATIENTS AND METHODS: 84 patients with controlled acromegaly after surgery (n=23) or SA (n=61) underwent a GH-profile including an OGTT, at baseline and after 12 months. SA patients were randomized to monitoring according to either IGF-I (n= 33) or GHnadir (n=28). SA dose escalation...

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

    Science.gov (United States)

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

    2017-03-01

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

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

    DEFF Research Database (Denmark)

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

    2014-01-01

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

  20. Pregnancy in a woman with acromegaly after transsphenoidal partial resection of pituitary macroadenoma - a case report.

    Science.gov (United States)

    Tomczyk, Katarzyna; Rzymski, Paweł; Woźniak, Jakub; Wilczak, Maciej

    2017-12-22

    Acromegaly is a systemic disease caused by an excessive release of growth hormone and the hypopituitarism, which is induced by macroadenoma local mass effect. The gynecological and obstetric disorders include irregular menstrual cycles, anovulatory cycles and infertility. Therefore, pregnancy in patients affected by the disease is rare. Patient described in this study became pregnant after pharmacological, surgical and infertility treatment. Hence, the following paper presents the course of pregnancy and the discussion of the impact of acromegaly on female fertility, pregnancy, and concerns related to the diagnosis and treatment.

  1. Features of carbohydrate metabolism and incretin secretion in patients with Cushing disease and acromegaly

    Directory of Open Access Journals (Sweden)

    Lubov V. Matchekhina

    2017-10-01

    Full Text Available Aim. This study aims to analyse the rhythm and levels of incretins and neuropeptides secretion in patients with Cushing disease (CD and acromegaly, and thus specify the pathogenesis of carbohydrate metabolism disturbances. Matherials and methods. In this study, 42 patients (mean age, 37.5 years with CD and acromegaly were enrolled. All patients were newly diagnosed with CD and acromegaly, and none had a history of previous drug therapy, radiotherapy or pituitary surgery. All patients underwent OGTT, during which glucose, glucagon, GLP-1, GLP-2, GIP and ghrelin were evaluated at 0, 30 and 120 min, respectively. Results. During OGTT, glucose levels were not significantly different between the groups. The relevance of pre-diabetes was higher in patients with CD. In these patients, while glucagon levels were substantially higher at all cut-off points than those in controls (р = 0.001, GIP secretion was slightly lower. The acromegaly group was characterised by an inverse rhythm of GIP secretion with no peak level at 30 min. In addition, GLP-1 levels were significantly higher in patients with CD (р = 0.047. Similarly, GLP-2 levels were also significantly higher in patients with CD than in those with acromegaly and controls (p = 0.001. Finally, ghrelin levels were significantly higher in patients with CD (р = 0.013 and acromegaly (р = 0.023. Conclusion. More pleiotropic actions of glucocorticoids can explain the higher relevance of carbohydrate metabolism disturbances in patients with CD. This can also be explained by higher levels of glucagon secretion, which do not depend on the type of carbohydrate metabolism disorder and are stimulated by a direct action of glucocorticoids on the glucagon receptor. GIP and GLP-1 secretion in patients with CD and acromegaly are characterised by the inverse rhythm with no peak levels, implying that these hormones do not play a crucial role in the development of carbohydrate disturbances in these patients. In

  2. Acromegaly: Role of Surgery in the Therapeutic Armamentarium

    Directory of Open Access Journals (Sweden)

    Gerardo Guinto

    2012-01-01

    Full Text Available Acromegaly is a complex disease that requires the intervention of a multidisciplinary team. The most frequent clinical manifestations are growing of distal parts of the body and some areas of the face. Patients may also present arterial hypertension, diabetes mellitus, colonic polyps, cardiomegaly, neurological and endocrine changes secondary to the presence of a GH-secreting tumor in pituitary or extrapituitary origin, or eutopic hypothalamic GHRH hypersecretion and peripheral GHRH hypersecretion. Surgery is the first treatment used for most patients, regardless of the cause. In the great majority of cases, pituitary tumor can be removed through a transsphenoidal approach. Craniotomy is reserved for those cases with giant tumors, particularly when they grow toward the middle or posterior cranial fossa. Best surgical results are obtained when the tumor is confined into the sella turcica or if it has a regular suprasellar extension. When the disease cannot be controlled with surgery, medical treatment is indicated. Somatostatin analogues are included as the first line of medication, followed by dopamine agonist and growth hormone receptors antagonists. Radiation therapy can be also indicated in two main forms for residual tumor with medically refractory patients: radiosurgery for small tumors or fractionated stereotactic radiotherapy for larger ones.

  3. The clinical presentation and biochemical diagnosis of acromegaly and gigantism.

    Science.gov (United States)

    Jialal, I; Nathoo, B C; Joubert, S; Asmal, A C; Pillay, N L

    1982-04-24

    Over a 5-year period 14 patients with acromegaly and gigantism were seen at the endocrine clinic of King Edward VIII Hospital: 9 were Blacks and 5 Indians; 8 of the patients were women. The mean age of the patients was 46 years. Surprisingly, only 2 patients complained of acral overgrowth. Symptomatology was varied and not characteristic of the condition. On examination all patients had unequivocal signs of soft-tissue and bony overgrowth, 64% had visual abnormalities and 50% hypertension. Radiologically, 88% showed an enlarged pituitary fossa. On biochemical investigation, the fasting levels of growth hormone (GH) were increased in 12 patients and during oral glucose tolerance tests, the GH levels in these 12 patients were not suppressed. One patient in whom the fasting GH level was not increased had progressed to the stage of panhypopituitarism, in the remaining patient challenge with thyrotrophin-releasing hormone (TRH) led to increased GH levels and L-dopa challenge resulted in a paradoxical decrease in GH levels. Seven patients with increased GH levels who were challenged with L-dopa showed the typical decrease in GH levels found in this condition; in 5 of these patients, challenged with TRH, GH levels increased. The findings emphasize that despite the ease of clinical diagnosis, appropriate biochemical investigations are necessary to confirm the exact status of the disease, which is rare in the population studied.

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

    Directory of Open Access Journals (Sweden)

    Cristina Alvarez-Escola

    2016-11-01

    Full Text Available In patients with active acromegaly after pituitary surgery, somatostatin analogues are effective in controlling the disease and can even be curative in some cases. After treatment discontinuation, the likelihood of disease recurrence is high. However, a small subset of patients remains symptom-free after discontinuation, with normalized growth hormone (GH and insulin-like growth factor (IGF1 levels. The characteristics of patients most likely to achieve sustained remission after treatment discontinuation are not well understood, although limited evidence suggests that sustained remission is more likely in patients with lower GH and IGF1 levels before treatment withdrawal, in those who respond well to low-dose treatment, in those without evidence of adenoma on an MRI scan and/or in patients who receive long-term treatment. In this report, we describe the case of a 56-year-old female patient treated with lanreotide Autogel for 11 years. Treatment was successfully discontinued, and the patient is currently disease-free on all relevant parameters (clinical, biochemical and tumour status. The successful outcome in this case adds to the small body of literature suggesting that some well-selected patients who receive long-term treatment with somatostatin analogues may achieve sustained remission.

  5. Clinical osteoarthritis predicts physical and psychological QoL in acromegaly patients

    NARCIS (Netherlands)

    Wassenaar, M. J. E.; Biermasz, N. R.; Kloppenburg, M.; van der Klaauw, A. A.; Tiemensma, J.; Smit, J. W. A.; Pereira, A. M.; Roelfsema, F.; Kroon, H. M.; Romijn, J. A.

    2010-01-01

    Quality of life is decreased in patients with long-term control of acromegaly. In addition, these patients suffer from irreversible osteoarthritis. The aim of this study was to assess the impact of joint-specific complaints, clinical and radiological signs of arthropathy on different aspects of

  6. Successful treatment of acromegaly in a diabetic cat with transsphenoidal hypophysectomy

    NARCIS (Netherlands)

    Meij, B.P.|info:eu-repo/dai/nl/164045805; Auriemma, E.|info:eu-repo/dai/nl/304834734; Grinwis, G.C.M.|info:eu-repo/dai/nl/141470909; Buijtels, J.J.C.W.M.|info:eu-repo/dai/nl/304830844; Kooistra, H.S.|info:eu-repo/dai/nl/205285864

    2010-01-01

    J Feline Med Surg. 2010 May;12(5):406-10. Successful treatment of acromegaly in a diabetic cat with transsphenoidal hypophysectomy. Meij BP, Auriemma E, Grinwis G, Buijtels JJ, Kooistra HS. Department of Clinical Sciences of Companion Animals, Faculty of Veterinary Medicine, Utrecht University,

  7. Glucose and Fat Metabolism in Acromegaly: From Mice Models to Patient Care.

    Science.gov (United States)

    Dal, Jakob; List, Edward O; Jørgensen, Jens Otto L; Berryman, Darlene E

    2016-01-01

    Patients with active acromegaly are frequently insulin resistant, glucose intolerant, and at risk for developing overt type 2 diabetes. At the same time, these patients have a relatively lean phenotype associated with mobilization and oxidation of free fatty acids. These features are reversed by curative surgical removal of the growth hormone (GH)-producing adenoma. Mouse models of acromegaly share many of these characteristics, including a lean phenotype and proneness to type 2 diabetes. There are, however, also species differences with respect to oxidation rates of glucose and fat as well as the specific mechanisms underlying GH-induced insulin resistance. The impact of acromegaly treatment on insulin sensitivity and glucose tolerance depends on the treatment modality (e.g. somatostatin analogs also suppress insulin secretion, whereas the GH antagonist restores insulin sensitivity). The interplay between animal research and clinical studies has proven useful in the field of acromegaly and should be continued in order to understand the metabolic actions of GH. © 2015 S. Karger AG, Basel.

  8. Structural tendon changes in patients with acromegaly: assessment of Achilles tendon with sonoelastography.

    Science.gov (United States)

    Onal, Eda Demil; Ipek, Ali; Evranos, Berna; Idilman, Ilkay Sedakat; Cakir, Bekir; Ersoy, Reyhan

    2016-03-01

    To describe the sonoelastographic appearance of the Achilles tendon in acromegalic patients and to determine whether the blood concentrations of growth hormone (GH) and insulin-like growth factor (IGF-1) are associated with the various sonographic elasticity types of Achilles tendons. Eighty-four Achilles tendons of 42 acromegaly patients and 84 Achilles tendons of 42 healthy volunteers were assessed with sonoelastography. The tendons were classified into two main types according to the elasticity features: type 1 blue/green (hard tissue) and type 2 yellow/red within green (intermediate-soft tissue). Two subtypes of these types were also defined. According to the definition, the elasticity of the tissue was in a spectrum ranging from hard to soft as the type progressed from 1a to 2b. The mean thickness of Achilles tendons in patients with acromegaly was significantly higher compared with healthy Achilles tendons (5.1+/-0.7 mm vs. 4.4+/-0.5, pacromegaly patients had type 2 sonoelastographic appearance of the Achilles tendon (124/252 third; 49.2% vs. 81/252 third; 32.1%, p=0.0001). Activity status of acromegaly and GH/IGF-I levels were similar in patients with different types of elasticity (p>0.05). Sonoelastography revealed structural changes in the tendinous tissue of patients with acromegaly, but it was not sensitive enough to reflect changes in the serum levels of GH/IGF-1.

  9. Pre- and Postoperative Body Composition and Metabolic Characteristics in Patients with Acromegaly: A Prospective Study

    Science.gov (United States)

    Gao, Lu; Li, Hailong; Wang, Qiang; Wang, Zihao

    2018-01-01

    Objective To investigate the preoperative body composition, metabolic characteristics, and postoperative changes in patients with active acromegaly and analyze the effects of gender and age. Methods We included 36 patients with untreated acromegaly and 37 patients with nonfunctional pituitary adenomas. Adipose tissue (AT), the visceral fat index (VFI), sclerotin, protein, skeletal muscle, total body water (TBW), intracellular water (ICW), and extracellular water (ECW) were measured using bioelectrical impedance analysis (BIA). Total energy expenditure (TEE) and basal metabolism (BM) were measured with a cardiopulmonary and metabolic analyzer (CMA). Tricep skinfold thickness (TST), bicep circumference, waistline, hipline, and calf circumference were measured with a skinfold caliper and tape. These indices were measured before surgery and 3 months and 1 year after surgery. Results Overall, AT, VFI, and TST were lower, whereas sclerotin, protein, skeletal muscle, TBW, ICW, ECW, TEE, and BM were higher in acromegaly patients. Postoperatively, TST rose initially and then decreased, the waistline increased, and sclerotin, skeletal muscle, TEE, and BM decreased. Changes in these indices differed with gender and age in unique patterns. Conclusions Body composition and metabolism in acromegaly patients changed after surgery, and gender and age influenced these changes. PMID:29531529

  10. Elevated serum IGF-1 level enhances retinal and choroidal thickness in untreated acromegaly patients.

    Science.gov (United States)

    Zhang, Xia; Ma, Jin; Wang, Yuhan; Li, Lüe; Gao, Lu; Guo, Xiaopeng; Xing, Bing; Zhong, Yong

    2018-03-01

    1) To compare the retinal, choroidal, Haller's layer, and Sattler's/choriocapillaris thicknesses of untreated acromegaly patients without chiasm compression or diabetes mellitus and healthy controls. 2) To evaluate the correlations of retinal and choroidal thicknesses with serum growth hormone (GH) and insulin-like growth factor 1 (IGF) burden. This prospective, case-control study included 27 untreated acromegaly patients and 27 sex-matched and age-matched controls. Subfoveal choroidal, Haller's layer and Sattler's/choriocapillaris thicknesses were determined by enhanced-depth imaging optical coherence tomography (EDI-OCT). Foveal and macular retinal thicknesses were determined with SD-OCT. GH and IGF-1 burdens were defined as the product of disease duration and treatment-naïve serum GH and IGF-1 levels. Compared with healthy controls, patients with acromegaly exhibited significantly increased foveal retinal (p = 0.003), subfoveal choroidal (p IGF-1 level (p = 0.03) and IGF-1 burden (p = 0.009). No significant correlations were detected between choroidal thickness and GH burden (p = 0.44). Retinal thickness was not significantly correlated with any factor. The choroidal thickness of acromegaly patients was greater than that of healthy controls and was significantly correlated with disease duration, IGF-1 level and IGF-1 burden, indicating that excessive serum IGF-1 and its exposure time have a combined effect on choroidal thickness.

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

    Science.gov (United States)

    Wang, Xian-Ling; Dou, Jing-Tao; Lü, Zhao-Hui; Zhong, Wen-Wen; Ba, Jian-Ming; Jin, Du; Lu, Ju-Ming; Pan, Chang-Yu; Mu, Yi-Ming

    2011-11-01

    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. Six patients with spontaneous remission of acromegaly or gigantism were enrolled. The clinical characteristics, endocrinological evaluation and imageological characteristics were retrospectively analyzed. 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. 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.

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

    Science.gov (United States)

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

    2017-04-01

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

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

    Science.gov (United States)

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

    2017-01-01

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

  14. Feline hypersomatotropism and acromegaly tumorigenesis: a potential role for the AIP gene.

    Science.gov (United States)

    Scudder, C J; Niessen, S J; Catchpole, B; Fowkes, R C; Church, D B; Forcada, Y

    2017-04-01

    Acromegaly in humans is usually sporadic, however up to 20% of familial isolated pituitary adenomas are caused by germline sequence variants of the aryl-hydrocarbon-receptor interacting protein (AIP) gene. Feline acromegaly has similarities to human acromegalic families with AIP mutations. The aim of this study was to sequence the feline AIP gene, identify sequence variants and compare the AIP gene sequence between feline acromegalic and control cats, and in acromegalic siblings. The feline AIP gene was amplified through PCR using whole blood genomic DNA from 10 acromegalic and 10 control cats, and 3 sibling pairs affected by acromegaly. PCR products were sequenced and compared with the published predicted feline AIP gene. A single nonsynonymous SNP was identified in exon 1 (AIP:c.9T > G) of two acromegalic cats and none of the control cats, as well as both members of one sibling pair. The region of this SNP is considered essential for the interaction of the AIP protein with its receptor. This sequence variant has not previously been reported in humans. Two additional synonymous sequence variants were identified (AIP:c.481C > T and AIP:c.826C > T). This is the first molecular study to investigate a potential genetic cause of feline acromegaly and identified a nonsynonymous AIP single nucleotide polymorphism in 20% of the acromegalic cat population evaluated, as well as in one of the sibling pairs evaluated. Copyright © 2016 Elsevier Inc. All rights reserved.

  15. Acromegaly discovered during a routine out-patient surgical procedure: a case report.

    Science.gov (United States)

    Chiaghana, Chukwudi O; Bauerfeind, Julia M; Sulek, Cheri A; Goldstein, J Christopher; Awoniyi, Caleb A

    2017-06-24

    Acromegaly is a rare syndrome in which there is unregulated hypersecretion of growth hormone. The anesthetic management of patients with this disorder is particularly challenging due to pre-existing cardiovascular and respiratory dysfunction, as well as recognized difficulties with airway management. Because of the insidious progression of the disease and the presence of nonspecific signs and symptoms, diagnosis is often made late when characteristic acromegalic features become apparent. We report the management of a 35-year-old African American man with previously undiagnosed acromegaly, who underwent a general anesthetic for same day surgery. Subtle physical features and difficult endotracheal intubation raised our suspicion for the diagnosis of acromegaly. Following an uncomplicated postoperative course he underwent workup for the disease, which was confirmed. In addition, brain magnetic resonance imaging showed a pituitary adenoma. A subsequent transsphenoidal hypophysectomy was performed successfully. This case underscores the notable absence of recognizing the clinical presentation of acromegaly in this patient by his primary care physician, and the value of thorough history taking, vigilance, and observation in making a new diagnosis that has the potential to alter a patient's health care and mitigate impending morbidity and/or mortality.

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

    International Nuclear Information System (INIS)

    Yarman, S.; Minareci, Oe.

    2004-01-01

    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.)

  17. Utility of baseline serum phosphorus levels for predicting remission in acromegaly patients.

    Science.gov (United States)

    Yalin, G Y; Tanrikulu, S; Gul, N; Uzum, A K; Aral, F; Tanakol, R

    2017-08-01

    High GH and IGF I levels increase tubular phosphate reabsorption in patients with acromegaly. We aimed to investigate the utility of serum phosphorus levels as an indicator for predicting chance of remission in acromegaly patients. Fifty-one patients (n: 51; F: 24, M: 27) with diagnosis of acromegaly were included in the study. Plasma IGF-1, Phosphorus (P) and nadir GH levels on oral glucose tolerance test (OGTT) at the time of diagnosis were analysed retrospectively. Patients were classified into two groups according to their plasma P levels; P ≤ 4.5 mg/dl (Group-1, n: 23, 45.1%), P > 4.5 mg/dl (Group-2, n: 28, 54.9%). Two groups were compared according to remission status; remission (n: 27) and non-remission (n: 24). Remission was defined with absence of clinical symptoms, normal plasma IGF-1 (adjusted for age and gender) and GH levels (acromegaly patients. Further studies with wider spectrum are needed to make specific suggestions.

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

    Energy Technology Data Exchange (ETDEWEB)

    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.)

  19. GH-secreting pituitary macroadenoma (acromegaly) associated with progressive dental malocclusion and refractory CPAP treatment.

    Science.gov (United States)

    Miranda-Rius, Jaume; Brunet-LLobet, Lluís; Lahor-Soler, Eduard; de Dios-Miranda, David; Giménez-Rubio, Josep Anton

    2017-05-10

    A link between progressive dental malocclusion, the use of a continuous positive airway pressure mask and GH-secreting pituitary macroadenoma (acromegaly) has not been previously reported. The present clinicopathological analysis stresses that tooth malposition should not be seen exclusively as a local process. A 62-year-old caucasian man with no relevant medical history reported difficulty chewing food and perceived voice alteration during his annual periodontal check-up. He also referred stiffness of the tongue, face, and submandibular area. The patient had been diagnosed with obstructive sleep apnea syndrome two years previously, since when he had worn a continuous positive airway pressure device during sleep. Exploration of the occlusion revealed significant changes: an atypical left lateral and anterior open bite with major buccoversion of teeth 33, 34, 35, 36. Inspection of the soft tissue revealed only macroglossia, although external palpation indicated a subcutaneous stiffness of the submandibular area. General analytical tests, including hormone profiles, and magnetic resonance imaging confirmed the diagnosis of acromegaly induced by a pituitary adenoma. Intrasellar tumor resection via transsphenoidal approach was performed. After surgery, the patient already noted a marked improvement of all symptoms associated with the acromegaly. Desaturation data also evolved favourably and the pulmonologist advised the patient to abandon the continuous positive airway pressure treatment. Progressive dental malocclusion may be associated with a systemic disease and the use of a nasal mask with premaxillary support may distort the diagnosis of acromegaly.

  20. Acromegaly is associated with increased cancer risk: a survey in Italy.

    Science.gov (United States)

    Terzolo, Massimo; Reimondo, Giuseppe; Berchialla, Paola; Ferrante, Emanuele; Malchiodi, Elena; De Marinis, Laura; Pivonello, Rosario; Grottoli, Silvia; Losa, Marco; Cannavo, Salvatore; Ferone, Diego; Montini, Marcella; Bondanelli, Marta; De Menis, Ernesto; Martini, Chiara; Puxeddu, Efisio; Velardo, Antonino; Peri, Alessandro; Faustini-Fustini, Marco; Tita, Patrizia; Pigliaru, Francesca; Peraga, Giulia; Borretta, Giorgio; Scaroni, Carla; Bazzoni, Nicoletta; Bianchi, Antonio; Berton, Alessandro; Serban, Andreea Liliana; Baldelli, Roberto; Fatti, Letizia Maria; Colao, Annamaria; Arosio, Maura

    2017-09-01

    It is debated if acromegalic patients have an increased risk to develop malignancies. The aim of the present study was to assess the standardized incidence ratios (SIRs) of different types of cancer in acromegaly on a large series of acromegalic patients managed in the somatostatin analogs era. It was evaluated the incidence of cancer in an Italian nationwide multicenter cohort study of 1512 acromegalic patients, 624 men and 888 women, mean age at diagnosis 45 ± 13 years, followed up for a mean of 10 years (12573 person-years) in respect to the general Italian population. Cancer was diagnosed in 124 patients, 72 women and 52 men. The SIRs for all cancers was significantly increased compared to the general Italian population (expected: 88, SIR 1.41; 95% CI, 1.18-1.68, P  acromegaly (all in women) did not change remarkably the study outcome. In multivariate analysis, the factors significantly associated with an increased risk of malignancy were age and family history of cancer, with a non-significant trend for the estimated duration of acromegaly before diagnosis. In conclusion, we found evidence that acromegaly in Italy is associated with a moderate increase in cancer risk. © 2017 Society for Endocrinology.

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

    Energy Technology Data Exchange (ETDEWEB)

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

    1992-01-01

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

  2. Therapeutic options in the management of acromegaly: focus on lanreotide Autogel

    NARCIS (Netherlands)

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

    2008-01-01

    In acromegaly, expert surgery is curative in only about 60% of patients. Postoperative radiation therapy is associated with a high incidence of hypopituitarism and its effect on growth hormone (GH) production is slow, so that adjuvant medical treatment becomes of importance in the management of many

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

    NARCIS (Netherlands)

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

    2015-01-01

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

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

    Science.gov (United States)

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

    2016-11-01

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

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

    Science.gov (United States)

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

    2016-06-01

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

  6. The Acylated/Unacylated Ghrelin Ratio Is Similar in Patients With Acromegaly During Different Treatment Regimens.

    Science.gov (United States)

    Muhammad, Ammar; Delhanty, Patric J D; Huisman, Martin; Visser, Jenny A; Jan van der Lelij, Aart; Neggers, Sebastian J C M M

    2017-07-01

    Data on plasma acylated ghrelin (AG) and unacylated ghrelin (UAG) levels in acromegaly are limited. High AG/UAG ratios are linked with type 2 diabetes, obesity, and hyperphagia (e.g., in Prader-Willi syndrome). To assess fasting plasma AG and UAG levels, and the AG/UAG ratio in acromegaly patients receiving combination treatment of long-acting somatostatin analogs (LA-SSAs) and pegvisomant (PEGV; n = 60). We used as controls acromegaly patients whose disease was controlled with PEGV monotherapy and medically naïve patients with active acromegaly. Fasting venous blood samples were collected and directly stabilized to inhibit deacylation of AG. Plasma AG and UAG levels were determined by double-antibody sandwich enzyme immunoassay, and the AG/UAG ratio was calculated. Plasma AG and UAG levels were significantly lower in patients with acromegaly receiving combination treatment [median, interquartile range (IQR): AG: 8.5 pg/mL, 2.9 to 21.1 pg/mL; UAG: 26.9 pg/mL, 11.2 to 42.1 pg/mL] compared with patients using PEGV alone [AG: 60.5 pg/mL (IQR, 58.8 to 77.4 pg/mL); UAG: 153.7 pg/mL (IQR, 127.3 to 196.0 pg/mL)] and medically naïve patients with acromegaly [AG: 24.0 pg/mL (IQR, 12.6 to 49.7 pg/mL); UAG: 56.3 pg/mL (IQR, 43.4 to 61.5 pg/mL)]. However, AG/UAG ratios were similar in all groups. Although plasma AG and UAG are suppressed during combination treatment with LA-SSAs and PEGV, the AG/UAG ratio remained similar. This shows that SSAs decrease both AG and UAG levels, which suggests that they do not alter metabolism significantly in acromegaly patients. Copyright © 2017 Endocrine Society

  7. Impact of exercise on quality of life and body-self perception of patients with acromegaly.

    Science.gov (United States)

    Hatipoglu, Esra; Topsakal, Nuri; Atilgan, Oya Erkut; Alcalar, Nilufer; Camliguney, Asiye Filiz; Niyazoglu, Mutlu; Cotuk, Hasan Birol; Kadioglu, Pinar

    2014-02-01

    In acromegaly the impact of therapy on well-being and self-perception of patients is not clearly defined. The data existing on the effect of treatment on health-related quality of life in patients with acromegaly is inconclusive. In this study we addressed the effect of exercise on health-related quality of life, symptoms of depression and perception of body image in patients with acromegaly. Patients with acromegaly were stratified into two groups according to their participation in a prescheduled program of exercise. Participants in the study group performed exercise for 75 min a day for 3 days a week during cosecutive 3 months. Warming, cardio, strength, balance and stretching moves applied in every course. Both the exercise group and control group were asked to complete a questionnaire on quality of life, symptoms of depression and self-perception of body image. Each questionnaire was answered by both groups before the beginning of the exercise program (at month-0) and after the completion of the program (at month-3). In exercise group after the completion of exercise period there was a tendency towards decreament in body mass index and IGF-I, although not statistically significant (p = 0.08 and p = 0.09). Self-assessment of body image improved significantly after participation in the exercise program (p = 0.01). Present findings support that exercise may be an adjunctive method for patients with acromegaly to improve self esteem and perception.

  8. Gigantism and acromegaly due to Xq26 microduplications and GPR101 mutation.

    Science.gov (United States)

    Trivellin, Giampaolo; Daly, Adrian F; Faucz, Fabio R; Yuan, Bo; Rostomyan, Liliya; Larco, Darwin O; Schernthaner-Reiter, Marie Helene; Szarek, Eva; Leal, Letícia F; Caberg, Jean-Hubert; Castermans, Emilie; Villa, Chiara; Dimopoulos, Aggeliki; Chittiboina, Prashant; Xekouki, Paraskevi; Shah, Nalini; Metzger, Daniel; Lysy, Philippe A; Ferrante, Emanuele; Strebkova, Natalia; Mazerkina, Nadia; Zatelli, Maria Chiara; Lodish, Maya; Horvath, Anelia; de Alexandre, Rodrigo Bertollo; Manning, Allison D; Levy, Isaac; Keil, Margaret F; Sierra, Maria de la Luz; Palmeira, Leonor; Coppieters, Wouter; Georges, Michel; Naves, Luciana A; Jamar, Mauricette; Bours, Vincent; Wu, T John; Choong, Catherine S; Bertherat, Jerome; Chanson, Philippe; Kamenický, Peter; Farrell, William E; Barlier, Anne; Quezado, Martha; Bjelobaba, Ivana; Stojilkovic, Stanko S; Wess, Jurgen; Costanzi, Stefano; Liu, Pengfei; Lupski, James R; Beckers, Albert; Stratakis, Constantine A

    2014-12-18

    Increased secretion of growth hormone leads to gigantism in children and acromegaly in adults; the genetic causes of gigantism and acromegaly are poorly understood. We performed clinical and genetic studies of samples obtained from 43 patients with gigantism and then sequenced an implicated gene in samples from 248 patients with acromegaly. We observed microduplication on chromosome Xq26.3 in samples from 13 patients with gigantism; of these samples, 4 were obtained from members of two unrelated kindreds, and 9 were from patients with sporadic cases. All the patients had disease onset during early childhood. Of the patients with gigantism who did not carry an Xq26.3 microduplication, none presented before the age of 5 years. Genomic characterization of the Xq26.3 region suggests that the microduplications are generated during chromosome replication and that they contain four protein-coding genes. Only one of these genes, GPR101, which encodes a G-protein-coupled receptor, was overexpressed in patients' pituitary lesions. We identified a recurrent GPR101 mutation (p.E308D) in 11 of 248 patients with acromegaly, with the mutation found mostly in tumors. When the mutation was transfected into rat GH3 cells, it led to increased release of growth hormone and proliferation of growth hormone-producing cells. We describe a pediatric disorder (which we have termed X-linked acrogigantism [X-LAG]) that is caused by an Xq26.3 genomic duplication and is characterized by early-onset gigantism resulting from an excess of growth hormone. Duplication of GPR101 probably causes X-LAG. We also found a recurrent mutation in GPR101 in some adults with acromegaly. (Funded by the Eunice Kennedy Shriver National Institute of Child Health and Human Development and others.).

  9. The incidence and prevalence of acromegaly, a nationwide study from 1955 through 2013.

    Science.gov (United States)

    Hoskuldsdottir, Gudrun Thuridur; Fjalldal, Sigridur Bara; Sigurjonsdottir, Helga Agusta

    2015-12-01

    Acromegaly is a rare disease with complications and increased mortality. The incidence and prevalence of acromegaly worldwide is not well known. To gather information on patients diagnosed with acromegly in Iceland over 59 years. Information was retrospectively gathered about patients diagnosed with acromegaly from 1955 through 2013. Incidence was calculated from the total Icelandic population. Information was gathered from medical records at Landspitali National University Hospital, Iceland, housing the only endocrine department in the country, at the largest hospital outside of Reykjavik (Sjúkrahúsið á Akureyri, Akureyri Hospital) and the largest private outpatient clinic in Reykjavik, where some of the patients received follow-up care. Further, information on patients were sought from all endocrinologists treating adult patients in Iceland. All patients diagnosed with acromegaly during the study period were included. Fifty-two patients (32 men) were diagnosed during the study period. The average age at diagnosis was 44.5 years. Nine patients had died. Symptoms had been present for more than 3 years in most cases. Twenty-five patients had hypertension (48 %). Follow up information was available for 48 patients, 63 % were considered cured after treatment. The incidence of acromegaly in Iceland during the study period was much higher than earlier reports have indicated. During the last 9 years of the study 7.7 patients were diagnosed per million per year. At diagnosis, 38 % had developed hypertension and 10 % were diagnosed during follow up. This indicates the importance of endocrine disorders in the aetiology of hypertension.

  10. Effects of active acromegaly on bone mRNA and microRNA expression patterns.

    Science.gov (United States)

    Belaya, Zhanna; Grebennikova, Tatiana; Melnichenko, Galina; Nikitin, Alexey; Solodovnikov, Alexander; Brovkina, Olga; Grigoriev, Andrey; Rozhinskaya, Liudmila; Lutsenko, Alexander; Dedov, Ivan

    2018-04-01

    To evaluate the response of bone to chronic long-term growth hormone (GH) and insulin-like growth factor-1 (IGF1) excess by measuring the expression of selected mRNA and microRNA (miR) in bone tissue samples of patients with active acromegaly. Case-control study. Bone tissue samples were obtained during transsphenoidal adenomectomy from the sphenoid bone (sella turcica) from 14 patients with clinically and biochemically confirmed acromegaly and 10 patients with clinically non-functioning pituitary adenoma (NFPA) matched by sex and age. Expression of genes involved in the regulation of bone remodeling was studied using quantitative polymerase chain reaction (qPCR). Of the genes involved in osteoblast and osteoclast activity, only alkaline phosphatase (ALP) mRNA was 50% downregulated in patients with acromegaly. GH excess caused increased expression of the Wnt signaling antagonists ( DKK1) and agonists ( WNT10B) and changes in the levels of miR involved in mesenchymal stem cell commitment to chondrocytes (miR-199a-5p) or adipocytes (miR-27-5p, miR-125b-5p, miR-34a-5p, miR-188-3p) P  Acromegaly had minimal effects on tested mRNAs specific to osteoblast or osteoclast function except for downregulated ALP expression. The expressions of miR known to be involved in mesenchymal stem cell commitment and downregulated TWIST1 expression suggest acromegaly has a negative effect on osteoblastogenesis. © 2018 European Society of Endocrinology.

  11. Muscle strength in patients with acromegaly at diagnosis and during long-term follow-up.

    Science.gov (United States)

    Füchtbauer, Laila; Olsson, Daniel S; Bengtsson, Bengt-Åke; Norrman, Lise-Lott; Sunnerhagen, Katharina S; Johannsson, Gudmundur

    2017-08-01

    Patients with acromegaly have decreased body fat (BF) and increased extracellular water (ECW) and muscle mass. Although there is a lack of systematic studies on muscle function, it is believed that patients with acromegaly may suffer from proximal muscle weakness despite their increased muscle mass. We studied body composition and muscle function in untreated acromegaly and after biochemical remission. Prospective observational study. Patients with acromegaly underwent measurements of muscle strength (dynamometers) and body composition (four-compartment model) at diagnosis ( n  = 48), 1 year after surgery ( n  = 29) and after long-term follow-up (median 11 years) ( n  = 24). Results were compared to healthy subjects. Untreated patients had increased body cell mass (113 ± 9% of predicted) and ECW (110 ± 20%) and decreased BF (67 ± 7.6%). At one-year follow-up, serum concentration of IGF-I was reduced and body composition had normalized. At baseline, isometric muscle strength in knee flexors and extensors was normal and concentric strength was modestly increased whereas grip strength and endurance was reduced. After one year, muscle strength was normal in both patients with still active disease and patients in remission. At long-term follow-up, all patients were in remission. Most muscle function tests remained normal, but isometric flexion and the fatigue index were increased to 153 ± 42% and 139 ± 28% of predicted values, respectively. Patients with untreated acromegaly had increased body cell mass and normal or modestly increased proximal muscle strength, whereas their grip strength was reduced. After biochemical improvement and remission, body composition was normalized, hand grip strength was increased, whereas proximal muscle fatigue increased. © 2017 European Society of Endocrinology.

  12. Calcium and Bone Turnover Markers in Acromegaly: A Prospective, Controlled Study.

    Science.gov (United States)

    Constantin, Tina; Tangpricha, Vin; Shah, Reshma; Oyesiku, Nelson M; Ioachimescu, Octavian C; Ritchie, James; Ioachimescu, Adriana G

    2017-07-01

    Acromegaly has been associated with calcium-phosphate and bone turnover alterations. Controlled studies of these interactions are sparse. To evaluate calcium and bone metabolism in active and treated acromegaly. We conducted a controlled, prospective study at a tertiary referral center. We studied 22 patients with acromegaly referred for surgical or medical therapy (ACM) and 22 with nonfunctioning pituitary adenomas referred for surgery (control). Calcium (serum and urine), phosphorus, parathyroid hormone (PTH), 25-hydroxy- and 1,25-dihydroxy-vitamin D, bone turnover markers [serum C-terminal telopeptide of type 1 collagen (CTX) and procollagen type 1 N-terminal propeptide (P1NP)], and cytokines [receptor activator of nuclear factor κB ligand (RANK-L) and osteoprotegerin (OPG)] at baseline and 3 to 6 months after treatment. At baseline, the ACM group had lower PTH levels than controls (36.3 ± 13.9 pg/mL vs 56.0 ± 19.9 pg/mL) and higher phosphorus (4.34 ± 0.71 mg/dL vs 3.55 ± 0.50 mg/dL) (P acromegaly, serum calcium (9.52 ± 0.43 mg/dL to 9.26 ± 0.28 mg/dL), phosphorus (4.34 ± 0.71 mg/dL to 3.90 ± 0.80 mg/dL), and CTX (0.91 ± 0.75 ng/mL to 0.63 ± 0.68 ng/mL) decreased, while PTH increased (36.3 ± 13.9 pg/mL to 48.9 ± 16.7 pg/mL) (P Acromegaly patients exhibited PTH-independent calcium-phosphate alterations and enhanced coupled bone formation and resorption. Within 6 months of treatment, bone resorption decreased, whereas RANK-L/OPG changes were inconsistent. Copyright © 2017 Endocrine Society

  13. Retina ganglion cell/inner plexiform layer and peripapillary nerve fiber layer thickness in patients with acromegaly.

    Science.gov (United States)

    Şahin, Muhammed; Şahin, Alparslan; Kılınç, Faruk; Yüksel, Harun; Özkurt, Zeynep Gürsel; Türkcü, Fatih Mehmet; Pekkolay, Zafer; Soylu, Hikmet; Çaça, İhsan

    2017-06-01

    Increased secretion of growth hormone and insulin-like growth factor-1 in acromegaly has various effects on multiple organs. However, the ocular effects of acromegaly have yet to be investigated in detail. The aim of the present study was to compare retina ganglion cell/inner plexiform layer (GCIPL) and peripapillary nerve fiber layer thickness (pRNFL) between patients with acromegaly and healthy control subjects using spectral domain optical coherence tomography (SD-OCT). This cross-sectional, comparative study included 18 patients with acromegaly and 20 control subjects. All participants underwent SD-OCT to measure pRNFL (in the seven peripapillary areas), GCIPL (in the nine ETDRS areas), and central macular thickness (CMT). Visual field (VF) examinations were performed using a Humphrey field analyzer in acromegalic patients. Measurements were compared between patients with acromegaly and control subjects. A total of 33 eyes of 18 patients with acromegaly and 40 eyes of 20 control subjects met the inclusion criteria of the present study. The overall calculated average pRNFL thickness was significantly lower in patients with acromegaly than in control subjects (P = 0.01), with pRNFL thickness significantly lower in the temporal superior and temporal inferior quadrants. Contrary to our expectations, pRNFL thickness in the nasal quadrant was similar between acromegalic and control subjects. The mean overall pRNFL thickness and superonasal, nasal, inferonasal, and inferotemporal quadrant pRNFL thicknesses were found to correlate with the mean deviation (MD) according to Spearman's correlation. However, other quadrants were not correlated with VF sensitivity. No significant difference in CMT values was observed (P = 0.6). GCIPL thickness was significantly lower in all quadrants of the inner and outer macula, except for central and inferior outer quadrants, in the acromegaly group than that in the control group (P acromegaly compared with that in control subjects

  14. Persistent negative illness perceptions despite long-term biochemical control of acromegaly: novel application of the drawing test.

    Science.gov (United States)

    Tiemensma, Jitske; Pereira, Alberto M; Romijn, Johannes A; Broadbent, Elizabeth; Biermasz, Nienke R; Kaptein, Adrian A

    2015-05-01

    Patients with acromegaly have persistent complaints despite long-term biochemical control. Drawings can be used to assess patients' perceptions about their disease. We aimed to explore the utility of the drawing test and its relation to illness perceptions and quality of life (QoL) in patients after long-term remission of acromegaly. A cross-sectional study was conducted to evaluate the utility of the drawing test. A total of 50 patients after long-term remission (mean±s.e.m., 16±1.2 years) of acromegaly were included in this study. Patients completed the drawing test (two retrospective drawings of their body perception before acromegaly and during the active phase of acromegaly, and one drawing on the current condition after long-term remission), Illness Perception Questionnaire-Revised, Physical Symptom Checklist, EuroQoL-5D, and AcroQoL. Patients perceived a dramatic change in body size during the active state of the disease compared with the healthy state before the awareness of acromegaly. Patients reported that their body did not completely return to the original proportions after long-term remission. In addition, larger drawings indicated more negative consequences (Pdrawings also indicated more impaired QoL, especially disease-specific QoL (all Pdrawing test, illness perceptions, and QoL. The drawing test appears to be a novel and relatively easy tool to assess the perception of patients after long-term remission of acromegaly. The assessment of drawings may enable health care providers to appreciate the perceptions of patients with long-term remission of acromegaly, and enable discussion of symptoms and remission. © 2015 European Society of Endocrinology.

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

    Directory of Open Access Journals (Sweden)

    Karel Van Praet

    2015-07-01

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

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

    Science.gov (United States)

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

    2017-02-01

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

  17. Silent somatotroph tumour revisited from a study of 80 patients with and without acromegaly and a review of the literature.

    Science.gov (United States)

    Chinezu, Laura; Vasiljevic, Alexandre; Trouillas, Jacqueline; Lapoirie, Marion; Jouanneau, Emmanuel; Raverot, Gérald

    2017-02-01

    Silent somatotroph tumours are growth hormone (GH) immunoreactive (IR) pituitary tumours without clinical and biological signs of acromegaly. Their better characterisation is required to improve the diagnosis. Twenty-one silent somatotroph tumours were compared to 59 somatotroph tumours with acromegaly. Tumours in each group were classified into GH and plurihormonal (GH/prolactin (PRL)/±thyroid-stimulating hormone (TSH)) and into densely granulated (DG) and sparsely granulated (SG) types. The two groups were then compared with regards to proliferation (Ki-67, p53 indexes and mitotic count), differentiation (expression of somatostatin receptors SSTR 2A -SSTR 5 and transcription factor Pit-1) and secretory activity (% of GH- and PRL-IR cells). The silent somatotroph tumours represented 2% of all tested pituitary tumours combined. They were more frequent in women than in men (P = 0.002), more frequently plurihormonal and SG (P acromegaly. They all expressed SSTR 2A , SSTR 5 and Pit-1. The plurihormonal (GH/PRL/±TSH) tumours were mostly observed in women (sex ratio: 3/1) and in patients who were generally younger than those with acromegaly (P acromegaly. A low secretory activity of these tumours might explain the normal plasma values for GH and insulin-like growth factor 1 (IGF1) and the absence of clinical signs of acromegaly. © 2017 European Society of Endocrinology.

  18. High levels of IGF-1 predict difficult intubation of patients with acromegaly.

    Science.gov (United States)

    Zhang, Yu; Guo, Xiaopeng; Pei, Lijian; Zhang, Zhuhua; Tan, Gang; Xing, Bing

    2017-08-01

    To investigate the characteristics of difficult intubation and identify novel efficient predictors in patients with acromegaly. Patients with either untreated acromegaly or non-functional pituitary adenomas were enrolled. Patients with acromegaly underwent hormone assays, upper airway computed tomography and magnetic resonance imaging examinations and preoperative overnight polysomnography. The modified Mallampati classification, mouth opening, neck circumference, and neck extension were assessed, and the Cormack-Lehane grades and the time of tracheal intubation were recorded. Patients with acromegaly had a higher incidence of difficult intubation (62.5%). The time of tracheal intubation was prolonged, the neck circumference was enlarged, and the neck extension was confined. In patients with acromegaly and difficult intubation, the insulin-like growth factor 1 levels and apnea/hypoxia index were significantly higher compared to patients without difficult intubation (1115.40 ± 253.73 vs. 791.67 ± 206.62 ng/ml, P = 0.020; 22.17 ± 23.25 vs. 2.47 ± 2.84, P = 0.026, respectively). The bilateral regression analysis revealed that high levels of insulin-like growth factor 1 were an independent risk factor for developing difficult intubation (p = 0.042, Exp B = 1.006). The modified Mallampati classification was positively correlated with apnea/hypoxia index and could be calculated using the following logarithmic equation: MMC = 0.2982 * ln (AHI) + 2.1836. In patients with acromegaly, neck movement is confined, the time of tracheal intubation is prolonged, and the neck circumference is enlarged, and these patients suffer from an increased incidence of difficult intubation (62.5%) during anesthesia induction. The apnea/hypoxia index and insulin-like growth factor 1 levels are both increased in acromegalic patients with difficult intubation, and elevated insulin-like growth factor 1 levels are an independent risk factor of difficult

  19. Case of a woman with acromegaly whose presenting complaint was prolonged post-partum amenorrhea.

    Science.gov (United States)

    Hara, Tomomi; Kanasaki, Haruhiko; Oride, Aki; Moriyama, Masayuki; Kyo, Satoru

    2016-10-01

    We report a case of a woman who was incidentally diagnosed with acromegaly after referral for prolonged post-partum amenorrhea. A 25-year-old woman, gravida 2 para 1, had a normal transvaginal delivery and breastfeeding had been discontinued more than a year after delivery. Thereafter, spontaneous menstruation did not restart and she underwent hormonal therapy. Subsequently, she was referred to our university hospital for prolonged amenorrhea. Hormonal examination revealed severe hypogonadotropic hypogonadism (luteinizing hormone 0.5 mIU/mL, follicle-stimulating hormone amenorrhea, which revealed mass lesions extending from the pituitary fossa to the suprasellar area with similar signal intensity as the gray matter. In addition, bitemporal hemianopsia was observed on campimetry. After further examination, the patient was diagnosed with acromegaly. © 2016 Japan Society of Obstetrics and Gynecology.

  20. Endocrinological outcomes following endoscopic and microscopic transsphenoidal surgery in 113 patients with acromegaly.

    Science.gov (United States)

    Sarkar, Sauradeep; Rajaratnam, Simon; Chacko, Geeta; Chacko, Ari George

    2014-11-01

    To describe outcomes and complications in patients undergoing transsphenoidal surgery for acromegaly using the 2010 consensus criteria for biochemical remission. Retrospective review of 113 treatment naïve patients who underwent transsphenoidal surgery with the endoscopic (n=66) and the endonasal microscopic technique (n=47). Cure was defined if the age and sex-adjusted IGF-1 level was normal and either the basal GH was transsphenoidal surgery did not differ significantly overall (28.8% versus 36.2%). On univariate analysis, a preoperative GH level Transsphenoidal surgery remains the first line of treatment for patients with acromegaly, but invasive adenomas will frequently require adjuvant therapy. Copyright © 2014 Elsevier B.V. All rights reserved.

  1. Acromegaly incidence, prevalence, complications and long-term prognosis: a nationwide cohort study

    DEFF Research Database (Denmark)

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

    2016-01-01

    sampled from the general population as a comparison cohort. Cox regression and hazard ratios (HRs) with 95% confidence intervals (95% CIs) were used. RESULTS: Mean age at diagnosis (48.7 years (CI: 95%: 47.2-50.1)) and annual incidence rate (3.8 cases/10(6) persons (95% CI: 3.6-4.1)) among the 405 cases...... (HR: 2.1 (95% CI: 1.6-2.6)). The complication risk was also increased before the diagnosis of acromegaly. Overall mortality risk was elevated (HR: 1.3 (95% CI: 1.0-1.7)) but uninfluenced by treatment modality. CONCLUSION: (i) The incidence rate and age at diagnosis of acromegaly have been stable over...

  2. Craniopharyngioma in a patient with acromegaly due to a pituitary macroadenoma

    Science.gov (United States)

    El-Bilbeisi, Hazem; Ghannam, Mohammad; Nimri, Caramella F.; Ahmad, Azmi T.

    2010-01-01

    We present the first reported case of a craniopharyngioma as a second primary tumor in a patient with acromegaly due to a growth hormone (GH)-secreting pituitary adenoma. The patient was lost for follow-up for 18 years after trans-sphenoidal pituitary surgery for a GH-secreting pituitary adenoma. She presented with headaches and decreased visual acuity, and showed unsuppressed GH in an oral glucose load test with high IGF-1 levels. Brain MRI showed a suprasellar cystic mass and the patient underwent surgery for cyst drainage resulting in postoperative improvement in her vision. Biopsy of the mass confirmed the diagnosis of a craniopharyngioma. We stress the need for close follow-up of patients with acromegaly with adequate control of GH and IGF-1 levels. PMID:20864785

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

    Directory of Open Access Journals (Sweden)

    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.

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

    Directory of Open Access Journals (Sweden)

    Joanna Elżbieta Malicka

    2016-09-01

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

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

    Directory of Open Access Journals (Sweden)

    Michael Anthony Mooney

    2016-07-01

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

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

    Directory of Open Access Journals (Sweden)

    Purnima Agarwal

    2016-01-01

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

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

    International Nuclear Information System (INIS)

    Bergh, Alfons C.M. van den; Hoving, Marjanke A.; Links, Thera P.; Dullaart, Robin P.F.; Ranchor, Adelita V.; Weeme, Cees A. ter; Canrinus, Alof A.; Szabo, Ben G.; Pott, Jan-Willem R.

    2003-01-01

    For diagnosing radiation optic neuropathy (RON) ophthalmological and imaging data were evaluated from 63 acromegalic patients, irradiated between 1967 and 1998. Two patients developed RON: one patient in one optic nerve 10 years and another patient in both optic nerves 5 months after radiation therapy. RON is a rare complication after external beam radiation therapy for acromegaly, which can occur after a considerable latency period

  8. The Birmingham pituitary database: auditing the outcome of the treatment of acromegaly.

    Science.gov (United States)

    Jenkins, D; O'Brien, I; Johnson, A; Shakespear, R; Sheppard, M C; Stewart, P M

    1995-11-01

    Reduction of GH concentrations in acromegalic subjects may improve the increased mortality associated with the condition. Audit of the biochemical outcome of the management of acromegaly is, therefore, important. (1) To audit the biochemical 'cure' rate of acromegalic patients treated by surgery and/or radiotherapy under the care of the South Birmingham Endocrine Clinic. (2) To assess the correlation between random or basal GH with IGF-I and nadir GH during an oral glucose tolerance test. Ascertainment of acromegalic patients from a pituitary database. Mode of therapy, pretreatment GH, pretreatment tumour size, post-treatment GH, post-treatment IGF-I and post-treatment nadir GH were recorded. Biochemical cure was defined as a most recent random or basal GH < 5 mU/l. Cure rates were determined. Eighty-nine acromegalic patients were identified as having received surgery and/or radiotherapy. In 35/89 (39%) the most recent GH was < 5 mU/l. The cure rate following surgery was 26/78 (33%). This was not significantly associated with tumour size, but was associated with pretreatment GH concentration (chi 2 = 7.1, 2d.f., P < 0.05). Random/basal GH showed a log-linear association with IGF-I, r = 0.72, and a linear association with nadir GH, r = 0.93. Biochemical cure of acromegaly was more strongly associated with pretreatment GH than with tumour size. Random/basal GH measurements are useful and convenient for the audit of treatment outcome in acromegaly. Ways of improving the biochemical outcome of acromegaly should be sought.

  9. Budget impact of pasireotide LAR for the treatment of acromegaly, a rare endocrine disorder.

    Science.gov (United States)

    Zhang, J J; Nellesen, D; Ludlam, W H; Neary, M P

    2016-01-01

    Acromegaly is a rare disorder characterized by the over-production of growth hormone (GH). Patients often experience a range of chronic comorbidities including hypertension, cardiac dysfunction, diabetes, osteoarthropathy, and obstructive sleep apnea. Untreated or inadequately controlled patients incur substantial healthcare costs, while normalization of GH levels may reduce morbidity and mortality rates to be comparable to the general population. To assess the 3-year budget impact of pasireotide LAR on a US managed care health plan following pasireotide LAR availability. Two separate economic models were developed: one from the perspective of an entire health plan and another from the perspective of a pharmacy budget. The total budget impact model includes costs of drug therapies and other costs for treatment, monitoring, management of adverse events, and comorbidities. The pharmacy cost calculator only considers drug costs. The total estimated budget impact associated with the introduction of pasireotide LAR is 0.31 cents ($0.0031) per member per month (PMPM) in the first year, 0.78 cents ($0.0078) in the second year, and 1.42 cents ($0.0142) in the third year following FDA approval. Costs were similar or lower from a pharmacy budget impact perspective. For each patient achieving disease control, cost savings from reduced comorbidities amounted to $10,240 per year. Published data on comorbidities for acromegaly are limited. In the absence of data on acromegaly-related costs for some comorbidities, comorbidity costs for the general population were used (may be under-estimates). The budget impact of pasireotide LAR is expected to be modest, with an expected increase of 1.42 cents PMPM on the total health plan budget in the third year after FDA approval. The efficacy of pasireotide LAR in acromegaly, as demonstrated in head-to-head trials compared with currently available treatment options, is expected to be associated with a reduction of the prevalence of

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

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    Cecilia Follin

    2016-08-01

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

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

    Science.gov (United States)

    Orosz, Andrea; Csajbók, Éva; Czékus, Csilla; Gavallér, Henriette; Magony, Sándor; Valkusz, Zsuzsanna; Várkonyi, Tamás T.; Nemes, Attila; Baczkó, István; Forster, Tamás; Wittmann, Tibor; Papp, Julius Gy.; Varró, András; Lengyel, Csaba

    2015-01-01

    Cardiovascular diseases, including ventricular arrhythmias are responsible for increased mortality in patients with acromegaly. Acromegaly may cause repolarization abnormalities such as QT prolongation and impairment of repolarization reserve enhancing liability to arrhythmia. The aim of this study was to determine the short-term beat-to-beat QT variability in patients with acromegaly. Thirty acromegalic patients (23 women and 7 men, mean age±SD: 55.7±10.4 years) were compared with age- and sex-matched volunteers (mean age 51.3±7.6 years). Cardiac repolarization parameters including frequency corrected QT interval, PQ and QRS intervals, duration of terminal part of T waves (Tpeak-Tend) and short-term variability of QT interval were evaluated. All acromegalic patients and controls underwent transthoracic echocardiographic examination. Autonomic function was assessed by means of five standard cardiovascular reflex tests. Comparison of the two groups revealed no significant differences in the conventional ECG parameters of repolarization (QT: 401.1±30.6 ms vs 389.3±16.5 ms, corrected QT interval: 430.1±18.6 ms vs 425.6±17.3 ms, QT dispersion: 38.2±13.2 ms vs 36.6±10.2 ms; acromegaly vs control, respectively). However, short-term beat-to-beat QT variability was significantly increased in acromegalic patients (4.23±1.03 ms vs 3.02±0.80, Pacromegaly in spite of unchanged conventional parameters of ventricular repolarization. This enhanced temporal QT variability may be an early indicator of increased liability to arrhythmia. PMID:25915951

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

    Science.gov (United States)

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

    2017-12-01

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

  13. Cognitive-behavioral therapy improves the quality of life of patients with acromegaly.

    Science.gov (United States)

    Kunzler, Lia Silvia; Naves, Luciana Ansaneli; Casulari, Luiz Augusto

    2018-06-01

    The delayed diagnosis, altered body image, and clinical complications associated with acromegaly impair quality of life. To assess the efficacy of the cognitive-behavioral therapy (CBT) technique "Think Healthy" to increase the quality of life of patients with acromegaly. This non-randomized clinical trial examined ten patients with acromegaly (nine women and one man; mean age, 55.5 ± 8.4 years) from a convenience sample who received CBT. The intervention included nine weekly group therapy sessions. The quality of life questionnaire the 36-Item Short Form Survey (SF-36) and the Beck Depression Inventory (BDI) were administered during the pre- and post-intervention phases. The Wilcoxon signed-rank test was performed to assess the occurrence of significant differences. According to the SF-36, the general health domain significantly improved (d'  = - 0.264; p = 0.031). The mental health domain improved considerably (d'  = - 1.123; p = 0.012). Physical functioning showed a non-significant trend toward improvement (d'  = - 0.802; p = 0.078), although four of the five patients who showed floor effects improved and remained at this level. Regarding emotional well-being, five patients showed floor effects and four improved, and the condition did not change among any of the four patients who showed ceiling effects. No significant changes were found with regard to the other domains. No significant differences in the BDI were found before or after the intervention. The technique presented herein effectively improved the quality of life of patients with acromegaly with different levels of disease activity, type, and treatment time.

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

    Science.gov (United States)

    Zoppoli, Gabriele; Bianchi, Federico; Bruzzone, Andrea; Calvia, Alessandro; Oneto, Caterina; Passalia, Caterina; Balleari, Enrico; Bedognetti, Davide; Ponomareva, Elena; Nazzari, Elena; Castelletti, Lara; Castellan, Lucio; Minuto, Francesco; Ghio, Riccardo; Ferone, Diego

    2012-06-01

    Polycythemia associated with acromegaly is usually caused by the systemic manifestations of the disease, such as sleep-apnea or concomitant erythropoietin-secreting kidney tumors. The recognition of underlying pathologies requires a thorough diagnostic process. We report a unique case of acromegaly with polycythemia, not caused by commonly described manifestations of the disease, and receding with octreotide therapy. The medical history of 141 acromegalic patients followed by the Endocrinology Unit of the San Martino University Hospital in Genoa has been also reviewed, together with the literature evidence for similar cases. The diagnostic workflow and 2-years follow-up of a 43-years old acromegalic, polycythemic man with a history of past smoking, moderate hypertension, and mental retardation are described. The hematological parameters of our cohort was retrospectively compared with those of a healthy, age/gender-related control group as well. Therapy with octreotide LAR, 20 mg i.m. q28d was begun soon after diagnosis of acromegaly in the polycythemic patient. Haematocrit level, hormonal setting, as well as pituitary tumor size and visual perimetry during treatment were recorded. Octreotide LAR treatment normalized hormonal alterations, as well as hematological parameters. Polycythemia has not recurred after 2 years of therapy. The median hemoglobin and hematocrit levels of the retrospectively analyzed cohort of acromegalic were significantly lower than normal ranges of a healthy, age/sex- related control population. In conclusions, polycythemia can be a direct, albeit rare, secondary manifestation of acromegaly, that must be considered during the diagnostic work-up of acromegalic patients presenting with such disorder.

  15. Assessment of Nasal Carriage of Staphylococcus Aureus and Axillar Flora in Patients With Acromegaly.

    Science.gov (United States)

    Gen, Ramazan; Horasan, Elif Şahin; Çinkir, Ümit; Sezer, Kerem; Akbay, Esen

    2017-05-01

    Recent study showed that patients with acromegaly have typical skin findings including increased sebum secretion, decreased transepidermal water loss, more alkaline, and colder skin surface correlated with serum growth hormone and insulin-like growth factor 1 levels. Different anatomic localizations and texture of the skin differ in bacterial concentrations.Nasal carriage of Staphylococcus aureus and axillar flora in patients with acromegaly was compared with normal population with regard to duration of acromegaly as well as the growth hormone and insulin-like growth factor 1 levels. This patient-control prospective study was conducted in university hospitals in Mersin, Turkey. The study consisted of 30 active acromegalic patients and 60 healthy adults who had no previously diagnosed chronic illness as a control group. A total of 90 volunteers were enrolled in this study; nasal and axillar cultures were obtained. Axillar and nasal specimens from anterior nares of the individuals were taken using sterile swabs. Nasal colonization of Staphylococcus aureus was 13.3% in acromegalic patients, but 43.4% in control group. This difference was statistically significant (P = 0.004). Patients and control group compared according to axillar cultures, the authors determined proteus colonization 16.7% in patients with acromegaly but no proteus colonization in control group. This result was statistically significant (P = 0.001). Proteus colonization was negatively correlated only with disease duration in acromegalic patients (P = 0.017). The authors demonstrated that compared with healthy subjects, acromegalic patients had low percentage of nasal carriage of Staphylococcus aureus and more gram-negative basili in the axillar flora. These nasal and axillar flora changes should be considered for prophylactic antibiotics use before surgery and ampiric antibiotics use after surgery.

  16. The role of combination medical therapy in the treatment of acromegaly.

    Science.gov (United States)

    Lim, Dawn Shao Ting; Fleseriu, Maria

    2017-02-01

    Uncontrolled acromegaly results in approximately 2-fold excess mortality. Pituitary surgery is first-line therapy, and medical treatment is indicated for persistent disease. While cabergoline and pegvisomant are used in select patients, somatostatin receptor ligands (SRLs) remain the cornerstone of medical treatment. Management of patients poorly responsive to SRLs is therefore, challenging. The purpose of this review is to highlight the options for combination medical therapy in the treatment of acromegaly, with an emphasis on efficacy and safety. All original articles/abstracts detailing combination medical therapy in acromegaly were identified from a PubMed search. Studies reviewed included retrospective and open-label prospective studies. While the combination of SRL and cabergoline was generally well tolerated, a lower baseline insulin-like growth factor-1 (IGF-1) level was the best predictor of efficacy; this combination may be most effective in patients with mildly elevated IGF-1. SRL-pegvisomant combination normalized IGF-1 in the majority of patients; continued efficacy despite individual drug dosing reduction was also reported. The risk of significant liver enzyme elevation was, however, higher than that reported with SRL monotherapy; close monitoring is recommended. Data on pegvisomant-cabergoline combination is limited, but this may be an option in the setting of SRL intolerance. Reports on temozolomide used in combination with other medical therapies in patients with aggressive GH-secreting tumors are also summarized. While more prospective, randomized controlled trials on long-term efficacy and safety are needed, combination medical therapy remains a treatment strategy that should be considered for acromegaly patients poorly responsive to SRLs.

  17. Decreasing mortality and changes in treatment patterns in patients with acromegaly from a nationwide study.

    Science.gov (United States)

    Esposito, Daniela; Ragnarsson, Oskar; Granfeldt, Daniel; Marlow, Tom; Johannsson, Gudmundur; Olsson, Daniel S

    2018-05-01

    New therapeutic strategies have developed for the management of acromegaly over recent decades. Whether this has improved mortality has not been fully elucidated. The primary aim was to investigate mortality in a nationwide unselected cohort of patients with acromegaly. Secondary analyses included time trends in mortality and treatment patterns. A total of 1089 patients with acromegaly were identified in Swedish National Health Registries between 1987 and 2013. To analyse time trends, the cohort was divided into three periods (1987-1995, 1996-2004 and 2005-2013) based on the year of diagnosis. Using the Swedish population as reference, standardized mortality ratios (SMRs) were calculated with 95% confidence intervals (CIs). Overall SMR was 2.79 (95% CI: 2.43-3.15) with 232 observed and 83 expected deaths. Mortality was mainly related to circulatory diseases (SMR: 2.95, 95% CI: 2.35-3.55), including ischemic heart disease (2.00, 1.35-2.66) and cerebrovascular disease (3.99, 2.42-5.55) and malignancy (1.76, 1.27-2.26). Mortality decreased over time, with an SMR of 3.45 (2.87-4.02) and 1.86 (1.04-2.67) during the first and last time period, respectively ( P  = .015). During the same time periods, the frequency of pituitary surgery increased from 58% to 72% ( P  acromegaly, mainly related to circulatory and malignant diseases. Although still high, mortality significantly declined over time. This could be explained by the more frequent use of pituitary surgery, decreased prevalence of hypopituitarism and the availability of new medical treatment options. © 2018 European Society of Endocrinology.

  18. PREVALENCE OF SLEEP DISORDERED BREATHING IN PATIENTS WITH NEWLY DIAGNOSED ACROMEGALY

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    U. A. Tsoy

    2014-01-01

    Full Text Available Background: Obstructive sleep disordered breathing or obstructive sleep apnea (OSA is the most common respiratory impairment in acromegaly. OSA is bound up with heightened cardiovascular mortality. Aim: Тo study frequency, features, and structure of sleep disordered breathing in patients with newly diagnosed acromegaly and to elucidate the factors influencing their development. Materials and methods: 38 patients (10 men, 28 women, median age 53 (28-76 years, median body mass index (BMI 29 (19.9-44.3 kg/m² with newly diagnosed acromegaly were recruited into the study. All subjects underwent full polysomnography (Embla N7000, Natus, USA and Remlogica software (USA. Results: Sleep disordered breathing was found in 28 (73.7% patients. OSA was revealed in all cases, in 11 (39.3% subjects it was mixed. In 10 (35.7% patients OSA was mild, in 8 (28.6% moderate, and in 10 (35.7% severe. BMI (р<0.01, disease duration (р=0.003, and insulin-like growth factor-1 (IGF-1 level (р=0.04 were different in patients without OSA and patients with moderate-to-severe OSA. No difference was found in sex (р=0.4, age (р=0.064, and growth hormone level (р=0.6. Frequency of arterial hypertension, diabetes mellitus, and other glucose metabolism impairments was the same in subjects without OSA and with severe-to-moderate OSA. Conclusion: All patients with newly diagnosed acromegaly should undergo polysomnography. BMI, disease duration, and IGF-1 level are significant risk factors for OSA development. Correlation OSA with arterial hypertension and glucose metabolism impairments needs to be further investigated.

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

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    A V Dreval’

    2013-03-01

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

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

    Science.gov (United States)

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

    2016-01-01

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

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

    Directory of Open Access Journals (Sweden)

    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

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

    Science.gov (United States)

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

    2015-12-01

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

  3. The association between biochemical control and cardiovascular risk factors in acromegaly.

    Science.gov (United States)

    Carmichael, John D; Broder, Michael S; Cherepanov, Dasha; Chang, Eunice; Mamelak, Adam; Said, Qayyim; Neary, Maureen P; Bonert, Vivien

    2017-03-09

    The study aim was to estimate the proportion of acromegaly patients with various comorbidities and to determine if biochemical control was associated with reduced proportion of cardiovascular risk factors. Data were from a single-center acromegaly registry. Study patients were followed for ≥12 months after initial treatment. Study period was from first to last insulin-like growth factor-I and growth hormone tests. Of 121 patients, 55% were female. Mean age at diagnosis was 42.4 (SD: 15.0). Mean study period was 8.8 (SD: 7.2) years. Macroadenomas were observed in 93 of 106 patients (87.7%), and microadenomas in 13 (12.3%). Initial treatment was surgery in 104 patients (86%), pharmacotherapy in 16 (13.2%), and radiation therapy in 1 (0.8%). Of 120 patients, 79 (65.8%) achieved control during the study period. New onset comorbidities (reported 6 months after study start) were uncommon (acromegaly compared to their controlled counterparts in this single-center registry. About a third of the patients remained uncontrolled after a mean of >8 years of treatment, demonstrating the difficulty of achieving control in some patients.

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

    Directory of Open Access Journals (Sweden)

    Cordes Uwe

    2010-03-01

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

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

    Science.gov (United States)

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

    2016-01-01

    ABSTRACT The aryl hydrocarbon receptor interacting protein (AIP) founder mutation R304* (or p.R304*; NM_003977.3:c.910C>T, p.Arg304Ter) identified in Northern Ireland (NI) predisposes to acromegaly/gigantism; its population health impact remains unexplored. We measured R304* carrier frequency in 936 Mid Ulster, 1,000 Greater Belfast (both in NI) and 2,094 Republic of Ireland (ROI) volunteers and in 116 NI or ROI acromegaly/gigantism patients. Carrier frequencies were 0.0064 in Mid Ulster (95%CI = 0.0027–0.013; P = 0.0005 vs. ROI), 0.001 in Greater Belfast (0.00011–0.0047) and zero in ROI (0–0.0014). R304* prevalence was elevated in acromegaly/gigantism patients in NI (11/87, 12.6%, P gigantism cases. tMRCA is consistent with historical/folklore accounts of Irish giants. Forward simulations predict many undetected carriers; geographically targeted population screening improves asymptomatic carrier identification, complementing clinical testing of patients/relatives. We generated disease awareness locally, necessary for early diagnosis and improved outcomes of AIP‐related disease. PMID:27650164

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

    Science.gov (United States)

    de Herder, Wouter W

    2009-01-01

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

  7. A rare challenging case of co-existent craniopharyngioma, acromegaly and squamous cell lung cancer.

    Science.gov (United States)

    Fountas, Athanasios; Chai, Shu Teng; Ayuk, John; Gittoes, Neil; Chavda, Swarupsinh; Karavitaki, Niki

    2018-01-01

    Co-existence of craniopharyngioma and acromegaly has been very rarely reported. A 65-year-old man presented with visual deterioration, fatigue and frontal headaches. Magnetic resonance imaging revealed a suprasellar heterogeneous, mainly cystic, 1.9 × 2 × 1.9 cm mass compressing the optic chiasm and expanding to the third ventricle; the findings were consistent with a craniopharyngioma. Pituitary hormone profile showed hypogonadotropic hypogonadism, mildly elevated prolactin, increased insulin-like growth factor 1 (IGF-1) and normal thyroid function and cortisol reserve. The patient had transsphenoidal surgery and pathology of the specimen was diagnostic of adamantinomatous craniopharyngioma. Post-operatively, he had diabetes insipidus, hypogonadotropic hypogonadism and adrenocorticotropic hormone and thyroid-stimulating hormone deficiency. Despite the hypopituitarism, his IGF-1 levels remained elevated and subsequent oral glucose tolerance test did not show complete growth hormone (GH) suppression. Further review of the pre-operative imaging revealed a 12 × 4 mm pituitary adenoma close to the right carotid artery and no signs of pituitary hyperplasia. At that time, he was also diagnosed with squamous cell carcinoma of the left upper lung lobe finally managed with radical radiotherapy. Treatment with long-acting somatostatin analogue was initiated leading to biochemical control of the acromegaly. Latest imaging has shown no evidence of craniopharyngioma regrowth and stable adenoma. This is a unique case report of co-existence of craniopharyngioma, acromegaly and squamous lung cell carcinoma that highlights diagnostic and management challenges. Potential effects of the GH hypersecretion on the co-existent tumours of this patient are also briefly discussed. Although an extremely rare clinical scenario, craniopharyngioma and acromegaly can co-exist; aetiopathogenic link between these two conditions is unlikely.Meticulous review of unexpected

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

    Science.gov (United States)

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

    2013-01-01

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

  9. Long-term treatment outcomes of acromegaly patients presenting biochemically-uncontrolled at a tertiary pituitary center.

    Science.gov (United States)

    Carmichael, John D; Broder, Michael S; Cherepanov, Dasha; Chang, Eunice; Mamelak, Adam; Said, Qayyim; Neary, Maureen P; Bonert, Vivien

    2017-08-04

    Acromegaly is a rare, slowly progressive disorder resulting from excessive growth hormone (GH) production by a pituitary somatotroph tumor. The objective of this study was to examine acromegaly treatment outcomes during long-term care at a specialized pituitary center in patients presenting with lack of biochemical control. Data came from an acromegaly registry at the Cedars-Sinai Medical Center Pituitary Center (center). Acromegaly patients included in this study were those who presented biochemically-uncontrolled for care at the center. Biochemical control status, based on serum insulin-like growth factor-1 values, was determined at presentation and at study end. Patient characteristics and acromegaly treatments were reported before and after presentation by presenting treatment status and final biochemical control status. Data on long-term follow-up were recorded from 1985 through June 2013. Seventy-four patients presented uncontrolled: 40 untreated (54.1%) and 34 (45.9%) previously-treated. Mean (SD) age at diagnosis was 43.2 (14.7); 32 (43.2%) were female patients. Of 65 patients with tumor size information, 59 (90.8%) had macroadenomas. Prior treatments among the 34 previously-treated patients were pituitary surgery alone (47.1%), surgery and medication (41.2%), and medication alone (11.8%). Of the 40 patients without prior treatment, 82.5% achieved control by study end. Of the 34 with prior treatment, 50% achieved control by study end. This observational study shows that treatment outcomes of biochemically-uncontrolled acromegaly patients improve with directed care, particularly for those that initially present untreated. Patients often require multiple modalities of treatment, many of which are offered with the highest quality at specialized pituitary centers. Despite specialized care, some patients were not able to achieve biochemical control with methods of treatment that were available at the time of their treatment, showing the need for additional

  10. PREDICTORS OF QUALITY OF LIFE IN 165 PATIENTS WITH ACROMEGALY: RESULTS FROM A SINGLE-CENTER STUDY.

    Science.gov (United States)

    Kreitschmann-Andermahr, Ilonka; Buchfelder, Michael; Kleist, Bernadette; Kohlmann, Johannes; Menzel, Christa; Buslei, Rolf; Kołtowska-Häggsträm, Maria; Strasburger, Christian; Siegel, Sonja

    2017-01-01

    Even if treated, acromegaly has a considerable impact on patient quality of life (QoL); despite this, the exact clinical determinants of QoL in acromegaly are unknown. This study retrospectively examines a cohort of treated patients with acromegaly, with the aim of identifying these determinants. Retrospective survey analysis, with 165 patients included in the study. All patients completed a survey, which included demographic data and the clinical details of their disease, the Short Form-36 Health Survey (SF-36), the revised Beck Depression Inventory (BDI-II), and the Bern Embitterment Inventory (BEI). Stepwise regression was used to identify predictors of QoL. The strongest predictors of the physical component score of the SF-36 were (in order of declining strength of association): Delay between first presentation of the disease and diagnosis, body mass index (BMI), number of doctors visited before the diagnosis of acromegaly, and age at diagnosis. For the mental component score, the strongest predictors were: number of doctors visited, previous radiotherapy, and age at study entry; and, for the BDI-II score: number of doctors visited, previous radiotherapy, age at study entry, and employment status at the time of diagnosis. The following were predictors of the BEI score: number of doctors visited, and age at study entry. Diagnostic delay and lack of diagnostic acumen in medical care provision are strong predictors of poor QoL in patients with acromegaly. Other identified parameters are radiotherapy, age, BMI, and employment status. An efficient acromegaly service should address these aspects when devising disease management plans. BDI-II = Beck Depression Inventory II BEI = Bern Embitterment Inventory BMI = body mass index IGF-1 = insulin-like growth factor 1 MCS = mental component summary (score) PCS = physical component summary (score) QoL = quality of life SDS = standard deviation score SF-36 = Short Form-36 Health Survey.

  11. Thyroid lesions in patients with acromegaly - case-control study and update to the meta-analysis.

    Science.gov (United States)

    Woliński, Kosma; Stangierski, Adam; Gurgul, Edyta; Bromińska, Barbara; Czarnywojtek, Agata; Lodyga, Martha; Ruchała, Marek

    2017-01-01

    Acromegaly results from oversecretion of growth hormone and subsequently insulin growth factor-1. According to some authors, the disease can cause increased prevalence of nodular goitre and thyroid cancer (TC). However, the number of studies comparing acromegalic patients with control groups is low. We aimed to assess the prevalence of thyroid lesions in patients with acromegaly in comparison to an age- and sex-matched control group and to update the meta-analysis previously performed in our department by the same authors. We searched medical documentation of patients with acromegaly treated in our department between 2003 and 2013. The prevalence of thyroid abnormalities was compared with the group of patients with hormonally inactive adrenal incidentalomas. To perform the meta-analytic part of the paper we also searched ten databases to find relevant papers. Two hundred and five patients with acromegaly and 184 patients with incidentalomas were included. Any thyroid lesions were present in 77.6% of patients with acromegaly vs. 63.0% with incidentalomas (p = 0.002), multinodular goitre - 66.8% vs. 47.8% (p = 0.0002), and TC- 5.4% vs. 2.7% (p = 0.21) respectively. For thyroid lesions the pooled odds ratio (OR) was 3.1 (95% confidence interval [CI] 1.8-5.5), and for TCs the OR was 4.5 (95% CI 1.9-10.3). According to our results thyroid lesions were significantly more common in patients with acromegaly; in case of TC the difference was not significant. The updated meta-analysis showed significantly increased prevalence of both disorders. In conclusion, systematic thyroid examination should be an important part of follow-up in case of acromegalic patients. (Endokrynol Pol 2017; 68 (1): 2-6).

  12. Prospective Study of Surgical Treatment of Acromegaly: Effects on Ghrelin, Weight, Adiposity, and Markers of CV Risk

    Science.gov (United States)

    Reyes-Vidal, Carlos; Fernandez, Jean Carlos; Bruce, Jeffrey N.; Crisman, Celina; Conwell, Irene M.; Kostadinov, Jane; Geer, Eliza B.; Post, Kalmon D.

    2014-01-01

    Context: Although epidemiological studies have found that GH and IGF-1 normalization reduce the excess mortality of active acromegaly to expected rates, cross-sectional data report some cardiovascular (CV) risk markers to be less favorable in remission than active acromegaly. Objective: The objective of the study was to test the hypothesis that remission of acromegaly after surgical therapy increases weight and adiposity and some CV risk markers and these changes are paralleled by a rise in ghrelin. Design: Forty-two adults with untreated, active acromegaly were studied prospectively. Changes in outcome measures from before to after surgery were assessed in 26 subjects achieving remission (normal IGF-1) and 16 with persistent active acromegaly (elevated IGF-1) after surgery. Setting: The study was conducted at tertiary referral centers for pituitary tumors. Main Outcome Measures: Endocrine, metabolic, and CV risk parameters, anthropometrics, and body composition by dual-energy X-ray absorptiometry were measured. Results: Remission increased total ghrelin, body weight, waist circumference, C-reactive protein, homocysteine, high-density lipoprotein, and leptin and reduced systolic blood pressure, homeostasis model assessment score, triglycerides, and lipoprotein (a) by 6 months and for 32 ± 4 months after surgery. The ghrelin rise correlated with the fall in the levels of GH, IGF-1, and insulin and insulin resistance. Weight, waist circumference, and ghrelin did not increase significantly in the persistent active acromegaly group. Total body fat, trunk fat, and perentage total body fat increased by 1 year after surgery in 15 remission subjects: the increase in body fat correlated with the rise in total ghrelin. Conclusions: Although most markers of CV risk improve with acromegaly remission after surgery, some markers and adiposity increase and are paralleled by a rise in total ghrelin, suggesting that these changes may be related. Understanding the mechanisms and

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

    Energy Technology Data Exchange (ETDEWEB)

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

    1993-08-01

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

  14. A rare challenging case of co-existent craniopharyngioma, acromegaly and squamous cell lung cancer

    Directory of Open Access Journals (Sweden)

    Athanasios Fountas

    2018-04-01

    Full Text Available Co-existence of craniopharyngioma and acromegaly has been very rarely reported. A 65-year-old man presented with visual deterioration, fatigue and frontal headaches. Magnetic resonance imaging revealed a suprasellar heterogeneous, mainly cystic, 1.9 × 2 × 1.9 cm mass compressing the optic chiasm and expanding to the third ventricle; the findings were consistent with a craniopharyngioma. Pituitary hormone profile showed hypogonadotropic hypogonadism, mildly elevated prolactin, increased insulin-like growth factor 1 (IGF-1 and normal thyroid function and cortisol reserve. The patient had transsphenoidal surgery and pathology of the specimen was diagnostic of adamantinomatous craniopharyngioma. Post-operatively, he had diabetes insipidus, hypogonadotropic hypogonadism and adrenocorticotropic hormone and thyroid-stimulating hormone deficiency. Despite the hypopituitarism, his IGF-1 levels remained elevated and subsequent oral glucose tolerance test did not show complete growth hormone (GH suppression. Further review of the pre-operative imaging revealed a 12 × 4 mm pituitary adenoma close to the right carotid artery and no signs of pituitary hyperplasia. At that time, he was also diagnosed with squamous cell carcinoma of the left upper lung lobe finally managed with radical radiotherapy. Treatment with long-acting somatostatin analogue was initiated leading to biochemical control of the acromegaly. Latest imaging has shown no evidence of craniopharyngioma regrowth and stable adenoma. This is a unique case report of co-existence of craniopharyngioma, acromegaly and squamous lung cell carcinoma that highlights diagnostic and management challenges. Potential effects of the GH hypersecretion on the co-existent tumours of this patient are also briefly discussed.

  15. Long-Term Endocrine Outcomes Following Endoscopic Endonasal Transsphenoidal Surgery for Acromegaly and Associated Prognostic Factors.

    Science.gov (United States)

    Babu, Harish; Ortega, Alicia; Nuno, Miriam; Dehghan, Aaron; Schweitzer, Aaron; Bonert, H Vivien; Carmichael, John D; Cooper, Odelia; Melmed, Shlomo; Mamelak, Adam N

    2017-08-01

    Long-term remission rates from endoscopic transsphenoidal surgery for acromegaly and their relationship to prognostic indicators of disease aggressiveness are not well documented. To investigate long-term remission rates in patients with acromegaly after endoscopic transsphenoidal surgery, and correlate this with molecular and radiographic markers of disease aggressiveness. We identified all patients undergoing endoscopic transsphenoidal surgery for acromegaly from 2005 to 2013 at Cedars-Sinai Pituitary Center. Hormonal remission was established by normal insulin-like growth factor (IGF)-1, basal serum growth hormone <2.5 ng/mL, and growth hormone suppression to <1 ng/mL following oral glucose tolerance test. Oral glucose tolerance test was performed at 3 months after surgery, and then as indicated. IGF-1 was measured at 3 months and then at least annually. We evaluated tumor granularity, nuclear expression of p21, Ki67 index, and extent of cavernous sinus invasion, and correlated these with remission status. Fifty-eight patients that underwent surgery had follow-up from 38 to 98 months (mean 64 ± 32.2 months). There were 21 microadenomas and 37 macroadenomas. Three months after surgery 40 of 58 patients (69%) were in biochemical remission. Four additional patients were in remission at 6 months after surgery, and 1 patient had recurrence within the first year after surgery. At last follow-up, 43 of 44 (74.1%) of patients remained in remission. Cavernous sinus invasion by tumor predicted failure to achieve remission. Prognostic markers of disease aggressiveness other than cavernous sinus invasion did not correlate with surgical outcome. Long-term remission after surgery alone was achieved in 74% of patients, indicating long-term efficacy of endoscopic surgery. Copyright © 2017 by the Congress of Neurological Surgeons

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

    Directory of Open Access Journals (Sweden)

    Victor Jacobus Geraedts

    2015-01-01

    Full Text Available IINTRODUCTION. Remission criteria of acromegaly are based on biochemical variables, i.e. normalization of increased hormone levels. However, the established reduction in Quality of Life (QoL is suggested to be independent of biochemical control. The aim of this study was to test which aspects predict Qol best in acromegaly. METHODS/Design. This is a prospective cohort study in 80 acromegalic patients, with a cross-sectional and longitudinal part. The main outcome measure was health-related quality of life (QoL, measured by a generic and a disease-specific questionnaire (the SF-36 and AcroQol. Main predictors were age, gender, biochemical control, disease characteristics, treatment modalities and psychopathology. RESULTS. Our cohort of 80 acromegalics had a mean age 54.7 ± 12.3 years with an average disease duration of 10.8 ± 10.0 years. Ratio macro-/microadenoma was 54/26. In adjusted mixed method models, we found that psychopathology significantly predicts QoL in acromegaly (in models including the variables age, gender, disease duration, tumor size, basal hormone levels, relevant treatment modalities and relevant comorbidities, with a higher degree of psychopathology indicating a lower QoL (depression vs. AcroQoL: B=-1.175, p<0.001, depression vs. SF36: B=-1.648, p<0.001, anxiety vs. AcroQoL: B=-0.399, p<0.001, anxiety vs. SF36: B=-0.661, p<0.001. The explained variances demonstrate superiority of psychopathology over biochemical control and other variables in predicting QoL in our models. DISCUSSION. Superiority of psychopathology over biochemical control calls for a more extensive approach regarding diagnosing depression and anxiety in pituitary adenomas to improve QoL. Depressive symptoms and anxiety are modifiable factors that might provide valuable targets for possible future treatment interventions.

  17. IGF-1 levels across the spectrum of normal to elevated in acromegaly: relationship to insulin sensitivity, markers of cardiovascular risk and body composition.

    Science.gov (United States)

    Reid, Tirissa J; Jin, Zhezhen; Shen, Wei; Reyes-Vidal, Carlos M; Fernandez, Jean Carlos; Bruce, Jeffrey N; Kostadinov, Jane; Post, Kalmon D; Freda, Pamela U

    2015-12-01

    Activity of acromegaly is gauged by levels of GH and IGF-1 and epidemiological studies demonstrate that their normalization reduces acromegaly's excess mortality rate. However, few data are available linking IGF-1 levels to features of the disease that may relate to cardiovascular (CV) risk. Therefore, we tested the hypothesis that serum IGF-1 levels relative to the upper normal limit relate to insulin sensitivity, serum CV risk markers and body composition in acromegaly. In this prospective, cross-sectional study conducted at a pituitary tumor referral center we studied 138 adult acromegaly patients, newly diagnosed and previously treated surgically, with fasting and post-oral glucose levels of endocrine and CV risk markers and body composition assessed by DXA. Active acromegaly is associated with lower insulin sensitivity, body fat and CRP levels than acromegaly in remission. %ULN IGF-1 strongly predicts insulin sensitivity, better than GH and this persists after adjustment for body fat and lean tissue mass. %ULN IGF-1 also relates inversely to CRP levels and fat mass, positively to lean tissue and skeletal muscle estimated (SM(E)) by DXA, but not to blood pressure, lipids, BMI or waist circumference. Gender interacts with the IGF-1-lean tissue mass relationship. Active acromegaly presents a unique combination of features associated with CV risk, reduced insulin sensitivity yet lower body fat and lower levels of some serum CV risk markers, a pattern that is reversed in remission. %ULN IGF-1 levels strongly predict these features. Given the known increased CV risk of active acromegaly, these findings suggest that of these factors insulin resistance is most strongly related to disease activity and potentially to the increased CV risk of active acromegaly.

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

    Directory of Open Access Journals (Sweden)

    Nikolaos Kyriakakis

    2017-01-01

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

  19. Transsphenoidal surgery in patients with acromegaly: operative strategies for overcoming technically challenging anatomical variations.

    Science.gov (United States)

    Zada, Gabriel; Cavallo, Luigi M; Esposito, Felice; Fernandez-Jimenez, Julio Cesar; Tasiou, Anastasia; De Angelis, Michelangelo; Cafiero, Tullio; Cappabianca, Paolo; Laws, Edward R

    2010-10-01

    In addition to difficulties with anesthetic and medical management, transsphenoidal operations in patients with longstanding acromegaly are associated with inherent intraoperative challenges because of anatomical variations that occur frequently in these patients. The object of this study was to review the overall safety profile and anatomical/technical challenges associated with transsphenoidal surgery in patients with acromegaly. The authors performed a retrospective analysis of 169 patients who underwent endoscopic transsphenoidal operations for growth hormone-secreting adenomas to assess the incidence of surgical complications. A review of frequently occurring anatomical challenges and operative strategies employed during each phase of the operation to address these particular issues was performed. Of 169 cases reviewed, there was no perioperative mortality. Internal carotid artery injury occurred in 1 patient (0.6%) with complex sinus anatomy, who remained neurologically intact following endovascular unilateral carotid artery occlusion. Other complications included: significant postoperative epistaxis (5 patients [3%]), transient diabetes insipidus (5 patients [3%]), delayed symptomatic hyponatremia (4 patients [2%]), CSF leak (2 patients [1%]), and pancreatitis (1 patient [0.6%]). Preoperative considerations in patients with acromegaly should include a cardiopulmonary evaluation and planning regarding intubation and other aspects of the anesthetic technique. During the nasal phase of the transsphenoidal operation, primary challenges include maintaining adequate visualization and hemostasis, which is frequently compromised by redundant, edematous nasal mucosa and bony hypertrophy of the septum and the nasal turbinates. During the sphenoid phase, adequate bony removal, optimization of working space, and correlation of imaging studies to intraoperative anatomy are major priorities. The sellar phase is frequently challenged by increased sellar floor thickness

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

    International Nuclear Information System (INIS)

    Clark, A.J.L.; Chahal, P.; Mashiter, K.; Joplin, G.F.

    1983-01-01

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

  1. Acromegaly in a domestic short-haired cat: First report from Iran

    Directory of Open Access Journals (Sweden)

    Mahdieh Rezaei

    2017-12-01

    Full Text Available A 12-year-old cat, weighting 7.4 kg, suffering from fatigue, exercise intolerance, polyphagia, polyuria and polydipsia was presented. Obesity, a massive head, inferior prognathia and widened inter-dental spaces were noted on examination. Radiographic surveys showed organs enlargement. Labaratory results revealed hyperglycemia and glycosuria. Based on fasting hyperglycemia, concurrent hyperglycemia and glycosuria, diagnosis of diabetes mellitus was made. However, according to the poor diabetic regulation, clinical signs and the absence of other diseases, a tentative diagnosis of acromegaly was confirmed by increased plasma levels of growth hormone. Managing diabetes mellitus with increasing doses of insulin was the only possible therapeutic strategy

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

    Energy Technology Data Exchange (ETDEWEB)

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

    1983-11-01

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

  3. Arthropathy in long-term cured acromegaly is characterised by osteophytes without joint space narrowing: a comparison with generalised osteoarthritis

    NARCIS (Netherlands)

    Wassenaar, M. J. E.; Biermasz, N. R.; Bijsterbosch, J.; Pereira, A. M.; Meulenbelt, I.; Smit, J. W. A.; Roelfsema, F.; Kroon, H. M.; Romijn, J. A.; Kloppenburg, M.

    2011-01-01

    To compare the distribution of osteophytes and joint space narrowing (JSN) between patients with acromegaly and primary generalised osteoarthritis to gain insight into the pathophysiological process of growth hormone (GH) and insulin-like growth factor type I (IGF-I)-mediated osteoarthritis. We

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

    Science.gov (United States)

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

    2016-04-01

    An increased prevalence of acromegaly was found some years ago in a highly polluted area in North-Eastern Sicily, where high concentration of nonmethane hydrocarbons, volatile organic compounds, and cadmium was found. Aryl hydrocarbon receptor (AHR) pathway has a key role in detoxification of these compounds and in tumorigenesis. We correlated the occurrence of AHR and/or AHR-interacting protein (AIP) gene variants with acromegaly severity according to pollution exposition. This was an observational, perspective study conducted over 7 years in four Italian referral centers for pituitary diseases in which 210 patients with acromegaly were enrolled between 2008 and 2015. Genetic screening of patients for AHR and AIP variants. Clinical, biochemical, and radiological data of patients with and without AIP and/or AHR gene variants, living in polluted (high-risk for health, [HR]) or nonpolluted (NP) areas of five Italian regions were evaluated and compared. Among the 23 patients from HR areas, nine showed AHR or AIP variants. Mean IGF-I levels and pituitary tumor diameter were significantly higher in these nine patients (HR/VAR+) than in the other 14 (HR/VAR−) and in the 187 from NP areas (44 NP/VAR+). Somatostatin analogs significantly decreased mean GH and IGF-I levels in patients from NP areas and in HR/VAR− (GH P acromegaly, increased pituitary tumor size, and somatostatin analog resistance in patients living in HR areas.

  5. Cardiac manifestations of GH deficiency after treatment for acromegaly: a comparison to patients with biochemical remission and controls

    NARCIS (Netherlands)

    van der Klaauw, Agatha A.; Bax, Jeroen J.; Bleeker, Gabe B.; Holman, Eduard R.; Delgado, V.; Smit, Johannes W. A.; Romijn, Johannes A.; Pereira, Alberto M.

    2008-01-01

    Both GH excess and GH deficiency (GHD) lead to specific cardiac pathology. The aim of this study was to evaluate cardiac morphology and function in patients with GHD after treatment for acromegaly. Cross-sectional study. Cardiac parameters were studied by conventional two-dimensional

  6. "Micromegaly": an update on the prevalence of acromegaly with apparently normal GH secretion in the modern era.

    Science.gov (United States)

    Butz, Laura B; Sullivan, Stephen E; Chandler, William F; Barkan, Ariel L

    2016-12-01

    Approximately 25 % of cases of clinically active acromegaly cases treated in our academic center between 1996 and 2000, were diagnosed in patients who had elevated plasma IGF-1 levels, but apparently "normal" 24-h mean plasma GH levels. The current study served to update the data for patients with acromegaly referred to our facility, after increasing awareness of this "normal" GH subpopulation throughout the medical community. A retrospective chart review was conducted on 157 patients with acromegaly who underwent resection of a confirmed somatotroph pituitary adenoma at the University of Michigan Health System between the dates of 1 Jan 2001 to 23 Sept 2015. Overall prevalence of acromegalic patients with "normal" GH levels, defined as GH 2 cm in the maximal diameter were encountered more frequently in the group with elevated GH (43 vs. 14 %, p acromegaly have "normal" GH, and therefore strengthens the growing body of evidence which supports the leading role of IGF-1 levels in diagnostic evaluation. At the present time, questions about the natural course of "micromegaly" and treatment benefits compared to the subpopulation with elevated GH levels remain unanswered, but research continues to build on our understanding of the heterogeneous population of individuals.

  7. Persistent negative illness perceptions despite long-term biochemical control of acromegaly: novel application of the drawing test

    NARCIS (Netherlands)

    Tiemensma, Jitske; Pereira, Alberto M.; Romijn, Johannes A.; Broadbent, Elizabeth; Biermasz, Nienke R.; Kaptein, Adrian A.

    2015-01-01

    Context and objective: Patients with acromegaly have persistent complaints despite long-term biochemical control. Drawings can be used to assess patients' perceptions about their disease. We aimed to explore the utility of the drawing test and its relation to illness perceptions and quality of life

  8. Conversion of daily pegvisomant to weekly pegvisomant combined with long-acting somatostatin analogs, in controlled acromegaly patients

    NARCIS (Netherlands)

    S.J.C.M.M. Neggers (Bas); W.W. de Herder (Wouter); R.A. Feelders (Richard); A-J. van der Lely (Aart-Jan)

    2011-01-01

    textabstractThe efficacy of combined treatment in active acromegaly with both long-acting somatostatin analogs (SRIF) and pegvisomant (PEG-V) has been well established. The aim was to describe the PEG-V dose reductions after the conversion from daily PEG-V to combination treatment. To clarify the

  9. Gender and age influence the relationship between serum GH and IGF-I in patients with acromegaly.

    Science.gov (United States)

    Parkinson, C; Renehan, A G; Ryder, W D J; O'Dwyer, S T; Shalet, S M; Trainer, P J

    2002-07-01

    In patients with acromegaly serum IGF-I is increasingly used as a marker of disease activity. As a result, the relationship between serum GH and IGF-I is of profound interest. Healthy females secrete three times more GH than males but have broadly similar serum IGF-I levels, and women with GH deficiency require 30-50% more exogenous GH to maintain the same serum IGF-I as GH-deficient men. In a selected cohort of patients with active acromegaly, studied off medical therapy using a single fasting serum GH and IGF-I measurement, we have reported previously that, for a given GH level, women have significantly lower circulating IGF-I. To evaluate the influence of age and gender on the relationship between serum GH and IGF-I in an unselected cohort of patients with acromegaly independent of disease control and medical therapy. Sixty (34 male) unselected patients with acromegaly (median age 51 years (range 24-81 years) attending a colonoscopy screening programme were studied. Forty-five had previously received pituitary radiotherapy. Patients had varying degrees of disease control and received medical therapy where appropriate. Mean serum GH was calculated from an eight-point day profile (n = 45) and values obtained during a 75-g oral glucose tolerance test (n = 15). Serum IGF-I, IGFBP-3 and acid-labile subunit were measured and the dependency of these factors on covariates such as log10 mean serum GH, sex, age and prior radiotherapy was assessed using regression techniques. The median calculated GH value was 4.7 mU/l (range 1-104). A significant linear association was observed between serum IGF-I and log10 mean serum GH for the cohort (R = 0.5, P fall by 0.37 nmol/l per year (P = 0.04, 95% CI 0.015-0.72). In keeping with previous observations of relative GH resistance in normal and GH-deficient females we have observed lower serum IGF-I levels for equivalent mean serum GH levels in females patients with acromegaly. This gender-dependent difference is independent of

  10. Cancer Incidence in Patients with Acromegaly: A cohort study and meta-analysis of the literature

    DEFF Research Database (Denmark)

    Dal, Jakob; Leisner, Michelle Z; Hermansen, Kasper

    2018-01-01

    -2010) including 529 acromegaly cases was performed. Incident cancer diagnoses and mortality were compared to national rates estimating standardized incidence ratios (SIRs). A meta-analysis of cancer SIRs from 23 studies (including the present one) was performed. Results: The cohort study identified 81 cases...... in acromegaly (SIR 1.3 [95% CI: 1.1-1.6]), cancer-specific mortality was not. The meta-analysis yielded a SIR of overall cancer of 1.5 [95% CI: 1.2-1.8]. SIRs were elevated for colorectal cancer: 2.6 [95% CI: 1.7-4.0], thyroid cancer: 9.2 [95% CI: 4.2-19.9], breast cancer: 1.6 [1.1-2.3], gastric cancer: 2.0 [95......% CI: 1.4-2.9], and urinary tract cancer: 1.5 [95% CI: 1.0-2.3]). In general, cancer SIR was higher in single-center studies and in studies with meta-analysis...

  11. The choice of treatment after incomplete adenomectomy in acromegaly: Proton - versus highvoltage radiation

    International Nuclear Information System (INIS)

    Luedecke, D.K.; Lutz, B.S.; Niedworok, G.

    1989-01-01

    The authors report the results of a study designed to compare the effectiveness of two different types of radiation in patients with acromegaly where surgical therapy had failed to normalize growth hormone(GH). Longterm follow-up after conventional high voltage radiation in 17 patients and protons therapy in 13 patients confirmed a similar reduction of GH levels in both groups. After 4,5 years a decrease of about 80% was achieved. After 'conventional radiation' GH was normal in 8(47%) and near normal in 6(35%) while proton therapy resulted in normalization in 5 and improvement in 5(38%). The slightly better results of 'conventional radiation' must be attributed to lower pretreatment levels of GH. Side effects as additional pituitary deficits and oculomotor palsies were more often seen after proton treatment. Since the results of both radiation methods are similar and proton therapy has a tendency to more serious side effects we recommend 'conventional radiation' as secondary treatment of acromegaly. (Authors)

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

    Directory of Open Access Journals (Sweden)

    Sang Ouk Chin

    2015-09-01

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

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

    Directory of Open Access Journals (Sweden)

    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.

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

    Directory of Open Access Journals (Sweden)

    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.

  15. An evaluation of the effects of somatostatin analogue therapy in non-functioning pituitary adenomas in comparison to acromegaly.

    Science.gov (United States)

    Zawada, Natalia Bożena; Kunert-Radek, Jolanta; Pawlikowski, Marek; Pisarek, Hanna; Radek, Maciej

    2016-01-01

    Non-functioning pituitary adenomas (NFPA) are often diagnosed late as invasive macroadenomas. The surgical resection is usually incomplete and about 50% of patients require additional surgery. Recent data suggest that somatostatin analogues (SSA), so important in the pharmacotherapy of acromegaly, can also be effective in the management of NFPA. We analysed data of patients who had been treated up to 10 years previously with SSA: 40 with acromegaly (23 - primary, 17 - recurrent tumours) and 22 with NFPA (4 - primary, 18 - recurrent tumours). Hormonal profile, dynamics of tumour size change, ophthalmic syndromes, somatostatin receptor (SSTR) scintigraphy, and immunohistochemistry of SSTR subtypes of operated tumours as well as side effects were investigated. Biochemical cure of acromegaly was achieved in 57.5% of patients, while reduction of tumour size was observed in 37% of patients and it was more frequent in not-operated cases. Regarding NFPA, stabilisation of tumour size was noticed in 68% of patients. Tumour shrinkage was reported in 9% of cases, but in 23% of the study group the adenoma size increased with indication for reoperation. The efficacy of SSA in NFPA is much lower in comparison to their well-established effects in the treatment of acromegaly. Stabilisation of tumour size, which is observed in the majority of NFPA, is significantly more frequent in comparison to the natural history of untreated NFPA and our previous studies as well. Analysis of SSTR subtypes is an argument in favour of introduction of novel broad-spectrum SSA that may be more effective in the treatment of NFPA. Referring to acromegaly, adenoma size decrease was reported more frequently in primary therapy. Considering recurrent tumours better outcomes were achieved in patients who were pre-treated with SSA before planned surgery. (Endokrynol Pol 2016; 67 (3): 292-298).

  16. The evaluation of central corneal thickness and intraocular pressure in conjunction with tear IGF-1 levels in patients with acromegaly.

    Science.gov (United States)

    Kan, Emrah; Kan, Elif K; Okuyucu, Ali

    2017-08-30

    To compare the central corneal thickness (CCT), intraocular pressure (IOP), and tear insulin-like growth factor 1 (IGF-1) levels between patients with acromegaly and a control group and to evaluate the possible effect of tear IGF-1 and duration of the disease on CCT and IOP. We included 31 patients with acromegaly (study group) and 40 age- and sex-matched controls in the study. Patients with acromegaly were divided into 2 subgroups based on disease status (active/inactive). All participants underwent complete ophthalmologic evaluation including CCT and IOP values. Basal tear samples were collected from both groups and tear IGF-1 levels were measured. The CCT, IOP, and tear IGF-1 levels were compared between groups and subgroups and the association between tear IGF-I levels and ocular parameters (CCT, IOP) and disease duration were also evaluated. Central corneal thickness, IOP, and tear IGF-1 levels did not show a significant difference between study and control groups. We also did not find a significant difference in terms of CCT, IOP, or tear IGF-1 levels between subgroups of patients. Correlation analysis did not show an association between the duration of disease and tear IGF-1 levels with CCT or IOP. There was no significant difference in tear IGF-1 levels between patients with acromegaly and controls. Additionally, there was no correlation between disease duration and tear IGF-1 levels with CCT or IOP levels. This lack of association may suggest that tear IGF-1 levels might not have an effect on CCT or IOP findings in patients with acromegaly.

  17. Gamma knife radiosurgery in patients with persistent acromegaly or Cushing's disease: long-term risk of hypopituitarism.

    Science.gov (United States)

    Cohen-Inbar, Or; Ramesh, Arjun; Xu, Zhiyuan; Vance, Mary Lee; Schlesinger, David; Sheehan, Jason P

    2016-04-01

    For patient with a recurrent or residual acromegaly or Cushing's disease (CD) after resection, gamma knife radiosurgery (GKRS) is often used. Hypopituitarism is the most common adverse effect after GKRS treatment. The paucity of studies with long-term follow-up has hampered understanding of the latent risks of hypopituitarism in patients with acromegaly or CD. We report the long-term risks of hypopituitarism for patients treated with GKRS for acromegaly or CD. From a prospectively created, IRB-approved database, we identified all patients with acromegaly or CD treated with GKRS at the University of Virginia from 1989 to 2008. Only patients with a minimum endocrine follow-up of 60 months were included. The median follow-up is 159·5 months (60·1-278). Thorough radiological and endocrine assessments were performed immediately before GKRS and at regular follow-up intervals. New onset of hypopituitarism was defined as pituitary hormone deficits after GKRS requiring corresponding hormone replacement. Sixty patients with either acromegaly or CD were included. Median tumour volume at time of GKRS was 1·3 cm(3) (0·3-13·4), and median margin dose was 25 Gy (6-30). GKRS-induced new pituitary deficiency occurred in 58·3% (n = 35) of patients. Growth hormone deficiency was most common (28·3%, n = 17). The actuarial overall rates of hypopituitarism at 3, 5 and 10 years were 10%, 21·7% and 53·3%, respectively. The median time to hypopituitarism was 61 months after GKRS (range, 12-160). Cavernous sinus invasion of the tumour was found to correlate with the occurrence of a new or progressive hypopituitarism after GKRS (P = 0·018). Delayed hypopituitarism increases as a function of time after radiosurgery. Hormone axes appear to vary in terms of radiosensitivity. Patients with adenoma in the cavernous sinus are more prone to develop loss of pituitary function after GKRS. © 2015 John Wiley & Sons Ltd.

  18. Characterization of transsphenoidal complications in patients with acromegaly: an analysis of inpatient data in the United States from 2002 to 2010.

    Science.gov (United States)

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

    2015-05-01

    Transsphenoidal surgery (TSS) is a common procedure for a variety of pituitary lesions. This procedure can be associated with complications related to the surgery or specific pathology. In this study, we evaluate inpatient postoperative complications among patients who underwent TSS for growth hormone adenomas using a nationally representative database, and compare patient characteristics and complications to patients who underwent TSS for other benign pituitary neoplasms. Analysis of the Nationwide Inpatient Sample revealed 13,070 TSS patients (including 892 with acromegaly) between 2002 and 2010. Complication rates, outcomes, patient demographics, hospital stay, and total charges were evaluated among TSS patients with and without acromegaly. There was an increase in TSS performed in both cohorts from 2002 to 2010. Acromegaly patients were younger, had shorter hospital stays, and incurred fewer charges. Acromegaly patients had a lower occurrence of postoperative urinary/renal complications (0.2% vs 1.1%), thromboembolic events (0% vs 0.4%), fluid/electrolyte abnormalities (5.7% vs 9.1%), and iatrogenic hypopituitarism (0.3% vs 1.1%) compared to other TSS patients (all p < 0.05). After adjusting for age, acromegalic patients maintained a statistically lower occurrence of fluid/electrolyte abnormalities (p = 0.007). Cerebrospinal fluid leak occurrence in acromegaly patients was 2.6% vs 1.7% in non-acromegaly patients, a result that did not reach significance (p = 0.054). Upon comparison of inpatient hospitalizations for patients undergoing TSS for growth hormone adenomas and other benign pituitary neoplasms, acromegaly patients had a significantly lower occurrence of postoperative fluid/electrolyte abnormalities. Acromegaly patients had shorter hospitalizations and subsequently fewer total charges. © 2015 ARS-AAOA, LLC.

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

    DEFF Research Database (Denmark)

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

    2010-01-01

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

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

    DEFF Research Database (Denmark)

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

    2010-01-01

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

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

    International Nuclear Information System (INIS)

    Ajaj, W.; Goyen, M.; Herrmann, B.; Massing, S.; Goehde, S.; Lauenstein, T.; Ruehm, S.G.

    2005-01-01

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

  2. 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

    Energy Technology Data Exchange (ETDEWEB)

    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.)

  3. More concerns and stronger beliefs about the necessity of medication in patients with acromegaly are associated with negative illness perceptions and impairment in quality of life.

    Science.gov (United States)

    Andela, Cornelie D; Biermasz, Nienke R; Kaptein, Adrian A; Pereira, Alberto M; Tiemensma, Jitske

    2015-10-01

    Patients with acromegaly can be treated with surgery, radiotherapy and/or medical treatment. In general, patients' beliefs about medication are associated with illness perceptions, a contributory factor of Quality of Life (QoL). At present, there are no quantitative studies on medication beliefs in patients with acromegaly. Here, we aimed to examine possible associations between medication beliefs, illness perceptions, and QoL. Furthermore we aimed to explore whether illness perceptions of patients with remission of acromegaly receiving medical treatment differ from patients without medical treatment. Cross-sectional evaluation of 73 patients with remission of acromegaly (n = 28 patients with medication, n = 45 without medication). The Beliefs about Medicines Questionnaire (BMQ), Illness Perception Questionnaire-Revised (IPQ-R), EuroQoL-5D, and AcroQoL were used for the assessment. Stronger beliefs about the necessity of medical treatment and stronger concerns about the adverse effects were associated with attributing more symptoms to acromegaly, perceiving more negative consequences, and having a stronger belief in a cyclical timeline (BMQ, all P Negative medication beliefs were related to more negative illness perceptions and worse disease-specific QoL. Patients receiving medical treatment for acromegaly tend to perceive a more chronic timeline of their disease, compared to patients with remission without medical treatment. These psychological factors need to be taken into account when treating patients and developing a psychosocial education program aiming to improve QoL. Copyright © 2015 Elsevier Ltd. All rights reserved.

  4. Five year remission of GHRH secreting bronchial neuroendocrine tumor with symptoms of acromegaly. Utility of chromogranin A in the monitoring of the disease

    International Nuclear Information System (INIS)

    Bolanowski, M.; Zatonska, K.; Kos-Kudla, B.; Rzeszutko, M.; Marciniak, M.

    2006-01-01

    Acromegaly is usually caused by excess GH (growth hormone) secretion by pituitary adenoma. Extremely rare (< 1% of cases) acromegaly can be a result of ectopic GHRH (growth hormone releasing hormone) secretion by bronchial tubes, lung, pancreatic or intestinal tumor. The aim of this description is to present the case of successfully treated acromegaly caused by ectopic GHRH secretion by bronchial neuroendocrine tumor and the usefulness of chromogranin A assay in the disease monitoring. The diagnosis of acromegaly in 61-year old woman was based on typical clinical picture and elevated GH and IGF-1(insulin-like growth factor-1) levels. MRI (magnetic resonance imaging) images revealed no tumor in the pituitary but only the pituitary enlargement. Moreover, the right lung tumor (10 cm size) and elevated GHRH level were documented. The secretion of GH, IGF-1 and GHRH were normalized and progression of acromegaly was stopped after the carcinoid tumor surgery. Currently, 5 year after surgery, acromegaly is still in the remission, as the normal levels of GH, IGF-1, chromogranin A and normal chest and pituitary images confirm. The authors emphasize usefulness of measurement of chromogranin A concentration for the evaluation of the tumor remission in case the routine GHRH assay is not accessible. (authors)

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

    Directory of Open Access Journals (Sweden)

    Bonert Vivien S

    2010-05-01

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

  6. Pure endoscopic transsphenoidal surgery for treatment of acromegaly: results of 67 cases treated in a pituitary center.

    Science.gov (United States)

    Gondim, Jackson A; Almeida, João Paulo; de Albuquerque, Lucas Alverne F; Gomes, Erika; Schops, Michele; Ferraz, Tania

    2010-10-01

    Acromegaly is a chronic disease related to the excess of growth hormone (GH) and insulin-like growth factor–I secretion, usually by pituitary adenomas. Traditional treatment of acromegaly consists of surgery, drug therapy, and eventually radiotherapy. The introduction of endoscopy as an additional tool for surgical treatment of pituitary adenomas and, therefore, acromegaly represents an important advance of pituitary surgery in the recent years. The aim of this retrospective study is to evaluate the results of pure transsphenoidal endoscopic surgery in a series of patients with acromegaly who were operated on by a pituitary specialist surgeon. The authors discuss the advantages, outcome, complications, and factors related to the success of the endoscopic approach in cases of GHsecreting adenomas. The authors retrospectively analyzed data from cases involving patients with GH-secreting adenomas who underwent pure transsphenoidal endoscopic surgery at the Department of Neurosurgery of the General Hospital in Fortaleza, Brazil, between 2000 and 2009. Tumors were classified according to size as micro- or macroadenomas, and tumor extension was analyzed based on suprasellar/parasellar extension and sella floor destruction. All patients were followed up for at least 1 year. The criteria of disease control were GH levels transsphenoidal surgery for treatment of acromegaly. Disease control was obtained in 50 cases (74.6%). The rate of treatment success was higher in patients with microadenomas (disease control achieved in 12 [85.7%] of 14 cases) than in those with larger lesions. Suprasellar/parasellar extension and high levels of sella floor erosion were associated with lower rates of disease control (p = 0.01 and p = 0.02, respectively). Complications related to the endoscopic surgery included epistaxis (6.0%), transitory diabetes insipidus (4.5%), and 1 case of seizure (1.5%). Endoscopic transsphenoidal surgery represents an effective option for treatment of patients

  7. Acromegaly according to the Danish National Registry of Patients: how valid are ICD diagnoses and how do patterns of registration affect the accuracy of registry data?

    Directory of Open Access Journals (Sweden)

    Dal J

    2014-09-01

    Full Text Available Jakob Dal,1 Nikolaj Skou,1 Eigil Husted Nielsen,2 Jens Otto Lunde Jørgensen,1 Lars Pedersen3 1Department of Endocrinology, Aarhus University Hospital, Aarhus, 2Department of Endocrinology, Aalborg University Hospital, Aalborg, 3Department of Clinical Epidemiology, Aarhus University Hospital, Aarhus, Denmark Background: The incidence of acromegaly is uncertain, since population-based studies are few. In the absence of a specific acromegaly registry, the Danish National Registry of Patients (DNRP becomes a potential source of data for studying the epidemiology of acromegaly, by linking all hospital discharge diagnoses to the personal identification numbers of individual Danish inhabitants. The validity of the DNRP with respect to acromegaly, however, remains to be tested. The aim of this study was to validate the International Classification of Diseases (ICD codes for acromegaly (ICD-8: 25300, 25301. ICD-10: E22.0 as used in the DNRP, and to assess the influence of various registration patterns on the accuracy of registry data. Methods: We identified patients registered with ICD codes for the diagnosis of acromegaly or other pituitary disorders during the period 1991–2009. Data on the institutional origin of each registration and the number of relevant DNRP registrations were recorded, and systematic patient chart reviews were performed to confirm the diagnosis. Results: In total, 110 cases of acromegaly were confirmed, compared with 275 registered cases, yielding a positive predictive value (PPV of 40%. When restricting the search to the regional highly specialized department of endocrinology, the PPV increased to 53% with no loss of cases with confirmed acromegaly. With a requirement of at least one, two, or three DNRP registrations, the PPV increased, but with a concurrent loss of confirmed cases. Conclusion: The DNRP seems to be a useful source for identifying new cases of acromegaly, especially when restricting the search to a relevant

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

    Science.gov (United States)

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

    2009-01-01

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

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

    Science.gov (United States)

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

    2016-02-01

    A 61-year-old man with obstructive sleep apnea syndrome and normal BMI complained of dyspnea. Nasofibroscopy revealed a global and major oedema of the glottis and supraglottis and also a paralysis of the left vocal fold. CT-scan pointed out a spontaneous hyperdensity of the left arytenoid cartilage. A tracheostomy was performed. Clinical examination revealed large hands and macroglossy with high IGF1 rate. MRI confirmed a supracentimetric pituitary adenoma. To our knowledge, this is the first description of a case of acute respiratory distress due to unilateral larynx paralysis leading to acromegaly diagnosis. This is due to submucosal hypertrophy and vocal cord immobility. Copyright © 2015 Elsevier Ireland Ltd. All rights reserved.

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

    Science.gov (United States)

    Tourtelot, John B; Vesely, David L

    2013-08-01

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

  11. The impact of transsphenoidal surgery on glucose homeostasis and insulin resistance in acromegaly.

    Science.gov (United States)

    Stelmachowska-Banaś, Maria; Zieliński, Grzegorz; Zdunowski, Piotr; Podgórski, Jan; Zgliczyński, Wocjiech

    2011-01-01

    Impaired glucose tolerance and overt diabetes mellitus are frequently associated with acro-megaly. The aim of this study was to find out whether these alterations could be reversed after transsphenoidal surgery. Two hundred and thirty-nine acromegalic patients were studied before and 6-12 months after transsphenoidal surgery. Diagnosis of active acromegaly was established on the basis of widely recognized criteria. In each patient, glucose and insulin concentrations were assessed during the 75 γ oral glucose tolerance test (OGTT). To estimate insulin resistance, we used homeostasis model assessment (HOMA-IR) and the quantitative insulin sensitivity check index (QUICKI). At the moment of diagnosis, diabetes mellitus was present in 25% of the acromegalic patients. After surgery, the pre-valence of diabetes mellitus normalized to the level present in the general Polish population. We found a statistically significant reduction after surgery in plasma glucose levels both fasting (89.45 ± 13.92 mg/dL vs. 99.12 ± 17.33 mg/dL, p surgery compared to the moment of diagnosis (15.44 ± 8.80 mIU/mL vs. 23.40 ± 10.24 mIU/mL, p transsphenoidal surgery, there was a significant reduction in HOMA-IR (3.08 vs. 6.76, p surgery in fasting glucose and insulin levels between patients with controlled and in-adequately controlled disease. We conclude that in acromegalic patients glucose homeostasis alterations and insulin sensitivity can be normalized after transsphenoidal surgery, even if strict biochemical cure criteria are not fulfilled.

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

    Science.gov (United States)

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

    2016-12-01

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

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

    International Nuclear Information System (INIS)

    Dietz, A.

    1982-01-01

    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.) [de

  14. Pasireotide: successful treatment of a sparsely granulated tumour in a resistant case of acromegaly

    Directory of Open Access Journals (Sweden)

    W K M G Amarawardena

    2017-07-01

    Full Text Available The granulation pattern of somatotroph adenomas is well known to be associated with differing clinical and biochemical characteristics, and it has been shown that sparsely granulated tumours respond poorly to commonly used somatostatin receptor ligands (SRLs. We report a challenging case of acromegaly with a sparsely granulated tumour resistant to multiple modalities of treatment, ultimately achieving biochemical control with pasireotide. A 26-year-old lady presented with classical features of acromegaly, which was confirmed by an oral glucose tolerance test. Insulin-like growth factor 1 (IGF1 was 1710 μg/L (103–310 μg/L and mean growth hormone (GH was >600 U/L. MRI scan showed a 4 cm pituitary macroadenoma with suprasellar extension and right-sided cavernous sinus invasion. She underwent trans-sphenoidal pituitary surgery. Histology displayed moderate amounts of sparsely granular eosinophilic cytoplasm, staining only for GH. Postoperative investigations showed uncontrolled disease (IGF1:1474 μg/L, mean GH:228 U/L and residual tumour in the cavernous sinus. She received external beam fractionated radiation. Over the years, she received octreotide LAR (up to 30 mg, lanreotide (up to 120 mg two weekly, cabergoline, pegvisomant and stereotactic radiosurgery to no avail. Only pegvisomant resulted in an element of disease control; however, this had to be stopped due to abnormal liver function tests. Fifteen years after the diagnosis, she was started on pasireotide 40 mg monthly. Within a month, her IGF1 dropped and has remained within the normal range (103–310 μg/L. Pasireotide has been well tolerated, and there has been significant clinical improvement. Somatostatin receptor subtyping revealed a positivity score of two for both sst5 and sst2a subtypes.

  15. EFFECT OF GROWTH HORMONE REPLACEMENT THERAPY ON THE QUALITY OF LIFE IN WOMEN WITH GROWTH HORMONE DEFICIENCY WHO HAVE A HISTORY OF ACROMEGALY VERSUS OTHER DISORDERS

    Science.gov (United States)

    Valassi, Elena; Brick, Danielle J.; Johnson, Jessica C.; Biller, Beverly M. K.; Klibanski, Anne; Miller, Karen K.

    2013-01-01

    Objective To compare the response in quality of life (QoL) to growth hormone (GH) replacement in women with GH deficiency (GHD) and a history of acromegaly with that in women with GHD of other causes. Methods Fifty-five women with GHD were studied: 17 with prior acromegaly and 38 with other causes of GHD. We compared two 6-month, randomized, placebo-controlled studies of GH therapy in women with hypopituitarism conducted with use of the same design—one in women with a history of acromegaly and one in women with no prior acromegaly. QoL was assessed with the following questionnaires: the QoL-Assessment of Growth Hormone deficiency in Adults (AGHDA), the Symptom Questionnaire, and the 36-Item Short-Form Health Survey (SF-36). Results The 2 groups had comparable mean pretreatment age, body mass index, and QoL scores and comparable mean GH dose at 6 months (0.61 ± 0.30 versus 0.67 ± 0.27 mg daily). After 6 months of GH replacement therapy, women with GHD and prior acromegaly demonstrated a greater improvement in AGHDA score, four SF-36 subscales (Role Limitations due to Physical Health, Energy or Fatigue, Emotional Well-Being, and Social Functioning), and the Somatic Symptoms subscale of the Symptom Questionnaire than did women with GHD of other causes. Poorer pretreatment QoL was associated with a greater improvement in QoL after administration of GH. Conclusion In this study, GH replacement therapy improved QoL in women with GHD and a history of acromegaly but not in women with GHD due to other hypothalamic and pituitary disorders. Further studies are needed to determine the long-term risks versus benefits of GH replacement in patients who develop GHD after definitive treatment for acromegaly. PMID:22440981

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

    Science.gov (United States)

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

    2011-03-01

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

  17. Computed tomography airway lumen volumetry in patients with acromegaly: Association with growth hormone levels and lung function.

    Science.gov (United States)

    Camilo, Gustavo Bittencourt; Carvalho, Alysson Roncally Silva; Guimarães, Alan Ranieri Medeiros; Kasuki, Leandro; Gadelha, Mônica Roberto; Mogami, Roberto; de Melo, Pedro Lopes; Lopes, Agnaldo José

    2017-10-01

    The segmentation and skeletonisation of images via computed tomography (CT) airway lumen volumetry provide a new perspective regarding the incorporation of this technique in medical practice. Our aim was to quantify morphological changes in the large airways of patients with acromegaly through CT and, secondarily, to correlate these findings with hormone levels and pulmonary function testing (PFT) parameters. This was a cross-sectional study in which 28 non-smoker patients with acromegaly and 15 control subjects underwent CT analysis of airway lumen volumetry with subsequent image segmentation and skeletonisation. Moreover, all participants were subjected to PFT. Compared with the controls, patients with acromegaly presented higher diameters in the trachea, right main bronchus and left main bronchus. The patients with acromegaly also showed a higher tracheal sinuosity index (the deviation of a line from the shortest path, calculated by dividing total length by shortest possible path) than the controls [1.06 (1.02-1.09) vs. 1.03 (1.02-1.04), P = 0.04], and tracheal stenosis was observed in 25% of these individuals. The tracheal area was correlated with the levels of growth hormone (r s  = 0.45, P = 0.02) and insulin-like growth factor type I (r s  = 0.38, P = 0.04). The ratio between the forced expiratory flow and forced inspiratory flow at 50% of the forced vital capacity was correlated with the tracheal area (r s  = 0.36, P = 0.02) and Δ tracheal diameters (r s  = 0.58, P volumetry, hormone levels and functional parameters of large airway obstruction. © 2017 The Royal Australian and New Zealand College of Radiologists.

  18. THE PREVALENCE OF COLONIC POLYPS IN PATIENTS WITH ACROMEGALY: A CASE-CONTROL, NESTED IN A COHORT COLONOSCOPIC STUDY.

    Science.gov (United States)

    Gonzalez, Baldomero; Vargas, Guadalupe; Mendoza, Victoria; Nava, Mariana; Rojas, Moisés; Mercado, Moisés

    2017-05-01

    Acromegaly is associated with an increased risk of colonic polyps. The magnitude of such risk is controversial, and the characteristics that distinguish patients who develop polyps from those who do not are not well established. This study was performed to determine the prevalence of colonic polyps upon the diagnosis of acromegaly and to compare the clinical and biochemical features of patients with and without polyps. Out of 165 patients who underwent a full colonoscopy upon diagnosis of acromegaly, 53 were found to harbor colonic lesions (cases), whereas the remaining 112 were used as controls. Demographic, clinical, and biochemical characteristics were compared between the 2 groups. The prevalence of colonic polyps was 32%, with an estimated relative risk of 6.21 (95% confidence interval [CI] 4.08-9.48). Adenomatous and nonadenomatous polyps were found in 22 and 31 patients, respectively. The most common location was the descending colon. Compared to patients without polyps, subjects with polyps were somewhat older and had significantly higher insulin-like growth factor-1 (IGF-1) levels and a higher prevalence of diabetes. Upon multivariate analysis, only IGF-1 level at diagnosis remained significantly associated with colonic polyps in general and with hyperplastic polyps in particular. Acromegaly is associated with an elevated risk of developing colonic polyps, particularly, distally located hyperplastic lesions. Except for a higher IGF-1 level at diagnosis, no distinctive clinical or biochemical features can be found among those who develop polyps compared to those who do not. CI = confidence interval GH = growth hormone IGF-1 = insulin-like growth factor 1 IQR = inter-quartile range RR = relative risk ULN = upper limit of normal.

  19. Growth hormone receptor exon 3 isoforms may have no importance in the clinical setting of multiethnic Brazilian acromegaly patients.

    Science.gov (United States)

    de Oliveira Machado, Evelyn; Lima, Carlos Henrique Azeredo; Ogino, Liana Lumi; Kasuki, Leandro; Gadelha, Mônica R

    2016-08-01

    Acromegaly is associated with significant morbidity and increased mortality, but has a variable severity phenotype. The presence of the exon 3-deleted isoform of the growth hormone receptor (d3-GHR) may influence the disease phenotype and treatment outcomes, including the frequency of biochemical discordance after medical treatment. The objective of this study was to analyze the influence of the d3-GHR isoform on clinical and biochemical characteristics and in the treatment outcomes of Brazilian multiethnic acromegaly patients. We retrospectively analyzed our acromegaly outpatient clinic databank and collected demographic, clinical, biochemical and treatment outcome data from those patients who agreed to participate in the study. A blood sample was collected from all patients, the DNA was extracted and the GHR isoforms were evaluated by PCR, with the full length (fl)-GHR represented by a 935-bp fragment and the d3-GHR represented by a 532-bp fragment. A total of 121 patients were included. Fifty-six patients (46.3 %) were full-length homozygous (fl/fl), 48 (39.7 %) were heterozygous (fl/d3) and 17 (14.0 %) were d3-GHR homozygous (d3/d3). There was no difference between patients homozygous for the fl isoform and those harboring at least one d3-GHR allele in the demographic, clinical and biochemical data or in the treatment outcomes, including somatostatin receptor ligands (SRL) monotherapy, combination therapy with SRL and cabergoline and pegvisomant treatment. There was also no difference between the groups for the frequency of GH and IGF-I discordance after medical treatment. GHR exon 3 genotyping appears to have no clinical significance, at least in Brazilian acromegaly patients.

  20. Effects of lanreotide Autogel primary therapy on symptoms and quality-of-life in acromegaly: data from the PRIMARYS study.

    Science.gov (United States)

    Caron, Philippe J; Bevan, John S; Petersenn, Stephan; Houchard, Aude; Sert, Caroline; Webb, Susan M

    2016-04-01

    To evaluate the effects of lanreotide Autogel on patient-reported outcomes and association with biochemical control, using PRIMARYS data. PRIMARYS was a 1-year, open-label study of lanreotide Autogel (Depot in USA) 120 mg every 4 weeks in 90 treatment-naïve patients with acromegaly. Symptoms were assessed using Patient-assessed Acromegaly Symptom Questionnaire (PASQ) and health-related quality of life (HRQoL) using the AcroQoL questionnaire. Correlations between PASQ and AcroQoL scores, and between PASQ/AcroQoL and growth hormone (GH)/insulin-like growth factor-1 (IGF-1) levels were also evaluated (post hoc). Acromegaly symptoms and HRQoL significantly improved from week 12 to week 48, with modest correlations at week 48 between PASQ total score (R = -0.55, p 50% of baseline standard deviation) in PASQ total score and >40% achieved a MID in AcroQoL global score (post hoc). Changes in PASQ scores were similar in biochemically controlled (GH levels ≤2.5 μg/L and normal IGF-1 levels) and uncontrolled groups, while changes in global and psychological AcroQoL scores were greater in the controlled group. There was no correlation between changes in PASQ or AcroQoL scores and changes in GH or IGF-1 levels. Primary treatment with lanreotide Autogel over 1 year was associated with rapid and sustained improvements in clinical signs and symptoms and HRQoL in patients with acromegaly. Improvements in HRQoL, but not symptoms, were greater in those achieving biochemical control (ClinicalTrials.gov: NCT00690898; EudraCT: 2007-000155-34).

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

    DEFF Research Database (Denmark)

    Andreassen, Mikkel; Faber, Jens; 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 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......) (95% CI 3-14), P = 0.007) and an increase in levels of BNP (median (ranges) 7 (0.58-286) vs. 20 (1-489) pg/mL, P = 0.033) and of NT-proBNP (63 (20-1004) vs. 80 (20-3391) pg/mL, P = 0.027). Assessed by the highly sensitive and precise CMRI method, 3 months treatment of acromegaly resulted...

  2. Safety and tolerability of pasireotide long-acting release in acromegaly-results from the acromegaly, open-label, multicenter, safety monitoring program for treating patients who have a need to receive medical therapy (ACCESS) study.

    Science.gov (United States)

    Fleseriu, Maria; Rusch, Elisha; Geer, Eliza B

    2017-01-01

    Pasireotide long-acting release is a somatostatin analog that is indicated for treatment of patients with acromegaly. This analysis documents the safety of pasireotide long-acting release in patients with acromegaly enrolled in the ACCESS trial (ClinicalTrials.gov identifier: NCT01995734). ACCESS is an open-label, multicenter, single-arm, expanded-treatment protocol designed to provide patients access to pasireotide long-acting release pending regulatory approval. Patients received pasireotide long-acting release 40 mg administered intramuscularly every 28 days. The primary outcome was the proportion of patients having a treatment-emergent grade ≥3 or serious adverse event. Efficacy data were not collected. Forty-four adult patients with active acromegaly were enrolled in the study for an average of 37.6 weeks (range, 4-70 weeks). Twenty-five grade ≥3 treatment-emergent adverse events were reported in 11 patients (25.0 %), 3 of whom (27.3 %) experienced grade ≥3 hyperglycemia. In patients treated with pasireotide long-acting release for ≥3 months (n = 42), mean glycated hemoglobin and fasting plasma glucose levels increased significantly from 5.9 % and 100.4 mg/dL at baseline to 6.8 % and 135.9 mg/dL at 3 months, respectively. Ten patients (22.7 %) were treated with pasireotide long-acting release for ≥15 months, after which mean glycated hemoglobin and fasting plasma glucose levels were 6.3 % and 123 mg/dL, respectively. Twenty-one patients (48 %) initiated antidiabetic medication. Grade ≥3 adverse events (primary outcome) were reported in 25.0 % of acromegaly patients treated with pasireotide long-acting release in a clinical setting. Hyperglycemia-related adverse events were reported in 45.5 % of patients, but were typically manageable, supporting the role of pasireotide long-acting release as a safe treatment option for acromegaly patients.

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

    Directory of Open Access Journals (Sweden)

    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.

  4. Usefulness of an ad hoc questionnaire (Acro-CQ) for the systematic assessment of acromegaly comorbidities at diagnosis and their management at follow-up.

    Science.gov (United States)

    Guaraldi, F; Gori, D; Beccuti, G; Prencipe, N; Giordano, R; Mints, Y; Di Giacomo, V S; Berton, A; Lorente, M; Gasco, V; Ghigo, E; Salvatori, R; Grottoli, S

    2016-11-01

    To determine the validity of a self-administered questionnaire (Acro-CQ) developed to systematically assess the presence, type and time of onset of acromegaly comorbidities. This is a cross-sectional study; 105 acromegaly patients and 147 controls with other types of pituitary adenoma, referred to a specialized Italian Center, autonomously compiled Acro-CQ in an outpatient clinical setting. To test its reliability in a different setting, Acro-CQ was administered via mail to 78 patients with acromegaly and 100 with other pituitary adenomas, referred to a specialized US Center. Data obtained from questionnaires in both settings were compared with medical records (gold standard). Demographics of patients and controls from both countries were similar. In both settings, >95 % of the questionnaires were completely filled; only one item was missed in the others. Concordance with medical record was excellent (k > 0.85) for most of the items, independently from the way of administration, patient age, gender and nationality, pituitary adenoma type and disease activity. Acro-CQ is an inexpensive, highly accepted from patients and reliable tool recommended to expedite systematic collection of relevant clinical data in acromegaly at diagnosis, to be replicated at follow-ups. This tool may guide a targeted, cost-effective management of complications. Moreover, it could be applied to retrieve data for survey studies in both acromegaly and other pituitary adenomas, as information is easily and rapidly accessible for statistical analysis.

  5. Treatment adherence and persistence with long-acting somatostatin analog therapy for the treatment of acromegaly: a retrospective analysis.

    Science.gov (United States)

    Gurel, Michelle H; Han, Yi; Stevens, Andrea L; Furtado, Aaron; Cox, David

    2017-04-04

    Many patients with acromegaly require medical treatment that includes somatostatin analogs (SSAs). Long-acting SSA formulations are widely used, due in part to increased patient convenience and increased treatment adherence vs daily medications. Although medication compliance can be poor in patients with chronic conditions, adherence and persistence with these SSAs in patients with acromegaly has not been evaluated. This analysis utilized claims data to estimate treatment adherence and persistence for lanreotide depot and long-acting octreotide in this population. This retrospective analysis used the MarketScan® database (~100 payors, 500 million claims in the US), which was searched between January 2007 and June 2012 to identify patients with acromegaly taking either lanreotide depot or long-acting octreotide. Patients switching treatments were excluded. Treatment adherence was assessed using medication possession ratio (MPR; number of doses dispensed in relation to dispensing period; ≥80% is considered adherent), injection count, and treatment time. Persistence was estimated by Kaplan-Meier analyses and Cox proportional hazards modeling. A washout period, defined as no acromegaly-related prescription activity 180 days prior to the index date, was employed to minimize effects of prior therapy and focus on patients more likely to be treatment-naïve. Altogether 1308 patients with acromegaly receiving a single SSA for treatment (1127 octreotide, 181 lanreotide) who had not switched treatments were identified. Mean MPR in patients with a 180-day washout (n = 663) was 89% for those receiving octreotide (n = 545) and 87% for those receiving lanreotide (n = 118). Median number of days on therapy was 169 (95% CI 135-232) for octreotide patients and 400 (95% CI 232-532) for lanreotide patients. The point estimate of the Cox proportional hazard ratio for stopping treatment was 1.385 for octreotide vs lanreotide (95% CI 1.079-1.777), suggesting a 38

  6. Intraoperative magnetic resonance imaging-assisted transsphenoidal pituitary surgery in patients with acromegaly.

    Science.gov (United States)

    Bellut, David; Hlavica, Martin; Schmid, Christoph; Bernays, René L

    2010-10-01

    Acromegaly is a rare disease, usually caused by a growth hormone (GH)-producing pituitary adenoma. If untreated, severe cardiovascular, metabolic, cosmetic, and orthopedic disturbances will result. Surgery is generally recommended as the first-line treatment. Transsphenoidal surgical techniques were recently extended by the introduction of intraoperative MR (iMR) imaging. In the present study, the contribution of ultra-low-field (0.15-T) iMR imaging to tumor resection, complication avoidance, and endocrinological and neurological outcome was analyzed. A series of 39 consecutive transsphenoidal iMR imaging-guided (using the PoleStar N20 device) surgical procedures performed between September 2005 and August 2009 for GH-producing pituitary adenomas was retrospectively analyzed. In addition to the patients' clinical data, the following criteria were evaluated independently: duration of surgery; length of hospital stay; endocrinological parameters; results of neurological examinations; and pre-, post-, and intraoperative MR imaging results. Thirty-seven patients with acromegaly underwent 39 transsphenoidal surgeries for pituitary adenomas. During a median follow-up period of 30 months (range 9-56 months), the remission rate was 73.5% in 34 patients with primary surgery and 20% in 5 cases with previous surgery; overall the remission rate was 66.7%. There were no serious postoperative complications. Detection of tumor remnant on iMR imaging led to a 5.1% increase in remission rate. In this largest study to date of GH-producing pituitary adenomas in which iMR imaging-guided transsphenoidal surgery was analyzed, the results suggest that this method is a highly effective and safe treatment modality, even compared with previously published surgical series in which high-field iMR imaging was used. Limitations of iMR imaging are the detection of small residual tumor in the cavernous sinus and persisting disease that could not be observed, even on diagnostic high-field follow

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

    DEFF Research Database (Denmark)

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

    2010-01-01

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

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

    DEFF Research Database (Denmark)

    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 bone and soft tissue turnover, as well as levels of PTH and vitamin D metabolites, in 16 patients (nine males; median age, 52 yr; range, 28-78 yr) with active acromegaly (serum IGF-I at least 30% above upper limit of an age-related reference range). Serum procollagen III amino-terminal propeptide...... (PIIINP) and type I procollagen amino-terminal propeptide, osteocalcin (OC), bone-related alkaline phosphatase, C-terminal cross-linked telopeptide of type I collagen (CTx), albumin-corrected calcium, intact PTH, 25-hydroxy vitamin D, 1,25-dihydroxy vitamin D [1,25-(OH)(2) vit D], urinary type 1 collagen...

  9. CLINICAL OUTCOMES AND SELF-REPORTED SYMPTOMS IN PATIENTS WITH ACROMEGALY: AN 8-YEAR FOLLOW-UP OF A LANREOTIDE STUDY.

    Science.gov (United States)

    Khairi, Shafaq; Sagvand, Babak Torabi; Pulaski-Liebert, Karen J; Tritos, Nicholas A; Klibanski, Anne; Nachtigall, Lisa B

    2017-01-01

    The aim of this study was to evaluate the proportion of patients with acromegaly who remained on long-term lanreotide depot after completion of an open-label multicenter phase III clinical trial (SALSA: A Multi Center Open Label Study to Assess the Ability of Subjects With Acromegaly or Their Partners to Administer Somatuline Autogel), compare baseline and long-term follow-up symptoms scores, and correlate scores with individual longitudinal clinical outcomes. Records of all subjects previously enrolled at the Massachusetts General Hospital site of SALSA were reviewed. Those who remained on lanreotide were interviewed and asked to complete a questionnaire that they had filled out in SALSA in 2007 regarding their current symptomatology and injection side effects, as well as to complete the Acromegaly Quality of Life Questionnaire. Furthermore, clinical, biochemical, and radiographic data related to acromegaly and its comorbidities were tracked throughout follow-up. Six out of 7 patients chose to remain on lanreotide, and 5 of them continued lanreotide depot through last follow-up, for up to 8 years or in 1 case until death. In all cases, lanreotide remained well tolerated, and insulin-like growth factor-1 levels and pituitary imaging remained well controlled on stable doses. While comorbidities persisted or developed, the self-reported symptom score after up to 8 years of therapy showed a significant decrease in frequency or resolution in symptoms that were reported at baseline. This study shows a significant decrease in frequency or resolution in self-reported symptoms in well-controlled patients receiving long-term lanreotide therapy. AcroQoL = Acromegaly Quality of Life Questionnaire GH = growth hormone GI = gastrointestinal IGF-1 = insulin-like growth factor-1 SALSA = A Multi Center Open Label Study to Assess the Ability of Subjects With Acromegaly or Their Partners to Administer Somatuline Autogel.

  10. Remission of acromegaly after treatment withdrawal in patients controlled by cabergoline alone or in combination with octreotide: results from a multicenter study.

    Science.gov (United States)

    Casagrande, A; Bronstein, M D; Jallad, R S; Mota, J I; Tabet, A; Abucham, J

    2017-05-01

    Remission of acromegaly has been reported after somatostatin analogs withdrawal, but not after withdrawal of combination therapy with cabergoline, and only in case reports of patients controlled by cabergoline alone. To establish the remission rates (normal IGF-1 for age/sex: IGF-1 ≤ 1.00 xULN) after withdrawal of combined treatment with octreotide LAR and cabergoline and of cabergoline alone, we prospectively studied 16 patients with acromegaly controlled by those treatments in the preceding 2 years as part of a larger study on remission of acromegaly after withdrawal of different medical treatments. Among 97 patients with controlled acromegaly included in the entire study, only 16 patients had been on combination therapy (n = 12) or cabergoline alone (n = 4). At 8 weeks after treatment withdrawal, three patients (19%) were in remission (short-term remission). At 60 weeks (long-term remission), IGF-1 levels were still in the normal range in two patients (12.5%) and remained normal up to 108 weeks after treatment withdrawal (last visit). One patient had been treated with cabergoline alone and another one with combination of octreotide and cabergoline before treatment withdrawal. Remission of acromegaly after treatment withdrawal seems to be uncommon in patients controlled by cabergoline, either as monotherapy or in combination with octreotide. In the future, larger studies and/or meta-analysis will be necessary to accurately establish the remission rates of acromegaly after withdrawal of cabergoline with or without somatostatin analogs.

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

    DEFF Research Database (Denmark)

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

    2001-01-01

    The aim was to evaluate, markers of disease activity in acromegaly in relation to perceived disease activity. Thirty-seven consecutively treated, acromegalic patients, classified by clinical symptoms as inactive (n=16), slightly active (n=10) and active (n=11), entered the study. When evaluating......-like growth factor binding protein-3 (IGFBP-3) with PV(pos) of 0.69 and 0.71 and PV(neg) of 0.91 and 0.92 respectively. We conclude that free IGF-I is more closely related than total IGF-I to perceived disease activity and is as such useful when evaluating previously treated acromegaly for disease activity...

  12. 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

    DEFF Research Database (Denmark)

    Skjaerbaek, Christian; Frystyk, Jan; Kaal, Andreas

    2000-01-01

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

  13. 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

    DEFF Research Database (Denmark)

    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...

  14. Long-term Primary Medical Therapy with Somatostatin Analogs in Acromegaly

    Directory of Open Access Journals (Sweden)

    Deng-Huang Su

    2006-01-01

    Full Text Available To cure acromegalic patients, transsphenoidal surgery is considered first, especially for microadenoma. However, less than 50% of patients with macroadenoma achieve satisfactory biochemical control. Moreover, surgery may cause hypopituitarism. Medical therapy may offer the prospect of near normalization of growth hormone (GH/insulin-like growth factor-1 levels with substantial tumor shrinkage in a significant number of patients. Here, we report two cases of acromegaly under treatment with somato-statin analogs alone for more than 10 years. Case 1 was a 54-year-old man with a pituitary macro-adenoma. He received 4 years of octreotide treatment followed by 6 years of prolonged-release (PR lanreotide resulting in normal GH level. Case 2 was a 60-year-old woman with a 1.3 cm pituitary tumor. She received 8 years of octreotide treatment followed by 6 years of PR lanreotide resulting in subnormal GH level and gallbladder sludge. She had received bilateral total hip replacement for hip osteoarthritis at the age of 59 years. These cases illustrate that long-term treatment with somatostatin analogs offers an alternative choice in selected acromegalic patients, such as those with pituitary tumor who cannot be cured by surgery, those who have unacceptable anesthetic risk and those who refuse surgery.

  15. Bioactivity and immunoactivity of growth hormone during dynamic testing of patients with acromegaly

    International Nuclear Information System (INIS)

    Baldwin, A.L.; Norman, M.; Butler, J.; Aston, R.; Buchanan, C.

    1988-01-01

    We have used the Nb2 cell proliferation bioassay for lactogenic hormones to investigate the biological activity of hGH in sera of patients with acromegaly. The specificity of the assay has been improved by the use of monoclonal antibodies to block the activity of individual lactogenic hormones. Disease activity in patients was assessed by scoring signs and symptoms, and by measuring IGF-I concentrations in some patients. Patients with a wide spectrum of disease activity were studied using a TRH test. hGH concentrations were measured by bioassay, RIA and immunoradiometric assay (IRMA) at 0,20 and 60 min after injection of TRH. There was a high degree of correlation between log 10 of all hGH concentrations measured by bioassay and RIA (r = 0.995, P<0.0001), between bioassay and IRMA (r = 0.990, P<0.0001), and between RIA and IRMA (r = 0.995, P<0.0001). In contrast to previous reports, we found no evidence for changes in the bioactivity of hGH secreted after pituitary stimulation. (author)

  16. [Carbohydrate metabolism in patients with acromegaly and Itsenko-Cushing disease].

    Science.gov (United States)

    Matchekhina, L V; Belaya, Zh E; Melnichenko, G A; Shestakova, M V

    2015-01-01

    The relevance of investigating carbohydrate metabolism (CM) in patients with acromegaly and Itsenko-Cushing disease is attributable to frequent glucose metabolic disturbances, on the one hand, and to difficulties in choosing sugar-lowering therapy in these categories of patients, on the other. The efficiency of hyperglycemia treatment in these patients may be reduced due to problems in achieving remission/cure of the underlying disease and to specific therapy favoring hyperglycemia. The top-priority tasks are to search for ways of reducing the frequency of CM abnormalities in patients with neuroendocrine diseases and to elaborate sugar-lowering therapy regimens. There is a growing interest in studies of the role of the incretin system in the pathogenesis of secondary hyperglycemias associated with neuroendocrine diseases. Nevertheless, few works have been published on this subject matter because of its novelty. There is a need for a further closer study of the specific features of incretin system function and the pharmacodynamics of incretin mimetics that are potential candidates as first-line drugs to treat secondary hyperglycemias. This paper attempts to summarize the available data obtained from studies into CM in neuroendocrine diseases.

  17. Long-term effects of pegvisomant on comorbidities in patients with acromegaly: a retrospective single-center study

    Science.gov (United States)

    Kuhn, Emmanuelle; Maione, Luigi; Bouchachi, Amir; Rozière, Myriam; Salenave, Sylvie; Brailly-Tabard, Sylvie; Young, Jacques; Kamenicky, Peter; Assayag, Patrick; Chanson, Philippe

    2015-01-01

    Context The effect of pegvisomant on IGF1 levels in patients with acromegaly is well documented, but little is known of its long-term impact on comorbidity. Aim The aim of this retrospective study was to evaluate the effects of long-term pegvisomant therapy on cardiorespiratory and metabolic comorbidity in patients with acromegaly. Patients and methods We analyzed the long-term (up to 10 years) effect of pegvisomant therapy given alone (n=19, 45%) or in addition to somatostatin analogues and/or cabergoline (n=23, 55%) on echocardiographic, polysomnographic and metabolic parameters in respectively 42, 12 and 26 patients with acromegaly followed in Bicêtre hospital. Results At the first cardiac evaluation, 20±16 months after pegvisomant introduction, IGF1 levels normalized in 29 (69%) of the 42 patients. The left ventricular ejection fraction (LVEF) improved significantly in patients whose basal LVEF was ≤60% and decreased in those whose LVEF was >70%. The left ventricular mass index (LVMi) decreased from 123±25 to 101±21 g/m2 (P<0.05) in the 17 patients with a basal LVMi higher than the median (91 g/m2), while it remained stable in the other patients. Pegvisomant reduced the apnoea–hypopnea index and cured obstructive sleep apnea (OSA) in four of the eight patients concerned. Long-term follow-up of 22 patients showed continuing improvements in cardiac parameters. The BMI and LDL cholesterol level increased minimally during pegvisomant therapy, and other lipid parameters were not modified. Conclusions Long-term pegvisomant therapy not only normalizes IGF1 in a large proportion of patients but also improves cardiac and respiratory comorbidity. PMID:26429918

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

    Energy Technology Data Exchange (ETDEWEB)

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

    2016-11-15

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

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

    Directory of Open Access Journals (Sweden)

    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.

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

    International Nuclear Information System (INIS)

    Shen, Ming; Zhang, Qilin; Liu, Wenjuan; Li, Yiming; Zhang, Zhaoyun; Ye, Hongying; He, Min; Lu, Bin; Yang, Yeping; Wang, Meng; Zhu, Jingjing; Ma, Zengyi; He, Wenqiang; Li, Shiqi; Shou, Xuefei; Qiao, Nidan; Ye, Zhao; Zhang, Yichao; Zhao, Yao; Wang, Yongfei; Yao, Zhenwei; Lu, Yun

    2016-01-01

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

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

    Science.gov (United States)

    Shen, Ming; Zhang, Qilin; Liu, Wenjuan; Wang, Meng; Zhu, Jingjing; Ma, Zengyi; He, Wenqiang; Li, Shiqi; Shou, Xuefei; Li, Yiming; Zhang, Zhaoyun; Ye, Hongying; He, Min; Lu, Bin; Yao, Zhenwei; Lu, Yun; Qiao, Nidan; Ye, Zhao; Zhang, Yichao; Yang, Yeping; Zhao, Yao; Wang, Yongfei

    2016-11-01

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

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

    NARCIS (Netherlands)

    Postma, Mark R; Netea-Maier, Romana T; van den Berg, Gerrit; Homan, Jens; Sluiter, Wim J; Wagenmakers, Margreet A; van den Bergh, Alfons C M; Wolffenbuttel, Bruce H R; Hermus, Ad R M M; van Beek, André P

    OBJECTIVE: To assess the influence of long-acting somatostatin analogs (SSTA) after initial pituitary surgery on long-term health-related quality of life (HR-QoL) in relation to disease control in patients with acromegaly. DESIGN: This is a cross-sectional study in two tertiary referral centers in

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

    NARCIS (Netherlands)

    Postma, M.R.; Netea-Maier, R.T.; Berg, G. van den; Homan, J.; Sluiter, W.J.; Wagenmakers, M.A.E.M.; Bergh, A.C. van den; Wolffenbuttel, B.H.R.; Hermus, A.R.M.M.; Beek, A.P. van

    2012-01-01

    OBJECTIVE: To assess the influence of long-acting somatostatin analogs (SSTA) after initial pituitary surgery on long-term health-related quality of life (HR-QoL) in relation to disease control in patients with acromegaly. DESIGN: This is a cross-sectional study in two tertiary referral centers in

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

    Science.gov (United States)

    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 octreotide LAR 20 mg at the time of enrolment. During 24 weeks of observation, AcroQoL survey questionnaires and measurement of growth hormone insulin-like growth factor 1(GH/IGF-I) were performed at baseline, week 12 and week 24. Main outcome measures We assessed the HRQoL of Korean patients with acromegaly after medical treatment with octreotide LAR using the validated Korean version of the AcroQoL questionnaire. Results Patients had a mean age of 47.2 years (29 males), and GH and IGF-I significantly decreased during the first 12 weeks (GH: 4.8 vs 1.9 μg/L, pacromegaly has a limited contribution to HRQoL as assessed by the AcroQoL. PMID:26063564

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

    DEFF Research Database (Denmark)

    Marina, Djordje; Burman, Pia; Klose, Marianne

    2015-01-01

    BACKGROUND: Somatotropinomas have unique "fingerprints" of somatostatin receptor (sst) expression, which are targets in treatment of acromegaly with somatostatin analogues (SSAs). However, a significant expression of sst is not always related to the biochemical response to SSAs. Headache is a com...

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

    NARCIS (Netherlands)

    W.W. de Herder (Wouter)

    2009-01-01

    textabstractIn 1886 Pierre Marie used the term "acromegaly" for the first time and gave a full description of the characteristic clinical picture. However several others had already given clear clinical descriptions before him and sometimes had given the disease other names. After 1886, it gradually

  7. Two phenotypes of arthropathy in long-term controlled acromegaly? A comparison between patients with and without joint space narrowing (JSN)

    NARCIS (Netherlands)

    Claessen, K. M. J. A.; Kloppenburg, M.; Kroon, H. M.; Romijn, J. A.; Pereira, A. M.; Biermasz, N. R.

    2013-01-01

    Arthropathy is an invalidating complication of acromegaly, also in long-term controlled patients, and is radiographically characterized by osteophytes and preserved joint spaces. However, joint space narrowing (JSN) is observed in the minority of patients. It is unknown whether JSN is the end-stage

  8. The predictive value of an acute octreotide suppression test in patients with acromegaly

    Directory of Open Access Journals (Sweden)

    Mateja Strinović

    2015-12-01

    Full Text Available Acromegaly is a rare chronic disorder that is in 95% of cases caused by growth hormone (GH secreting pituitary tumors. Endonasal transphenoidal surgery is usually considered the first line of treatment, followed by medical therapy for residual disease. The long-acting somatostatin (SA analogues have an important role in the medical treatment of these patients. SA exert their biological effects by activating somatostatin receptors (SSTR. The predominant types of SSTR receptors in GH-secreting pituitary tumors are subtypes 2 (SSTR2 and 5 (SSTR5. The efficacy of somatostatin analog therapy (SAT is determined by its effect on tumor shrinkage which is reported to be between 20 and 50%. Approximately 10-30% of GH-secreting pituitary tumors are resistant to SA because of variable or reduced tumoral expression of SSTR2 or SSTR5. In many centres a test dose (TD of octreotide is administered before commencing SAT in order to predict the long term response to treatment. There has been a great interest in identifying factors that predict a good response to SAT. To date, several studies that have examined the relationship between the TD of octreotide and SAT. It is crucial how we define response during the OST. Nadir of GH to 5mU/l or less during the OST has an excelent predictive value for evaluation of long-term response to SAT. On the other hand, if we define response as percentage of change of GH from it’s baseline values, then the OST is not usefull diagnostic test.

  9. Impaired quality of life in patients with treated acromegaly despite long-term biochemically stable disease: Results from a 5-years prospective study.

    Science.gov (United States)

    Kyriakakis, Nikolaos; Lynch, Julie; Gilbey, Stephen G; Webb, Susan M; Murray, Robert D

    2017-06-01

    Patients with acromegaly demonstrate impaired quality of life (QoL), but data on long-term QoL changes in treated acromegaly are limited. This study evaluates and identifies factors that influence QoL in patients with long-term biochemical remission. The study consists of a cross-sectional arm comparing QoL between patients with treated and controlled acromegaly and healthy controls; and a longitudinal arm assessing QoL changes in patients with biochemically stable disease during 5.7±0.6 years of follow-up. A total of 58 patients and 116 matched controls were recruited for the cross-sectional arm; 28 patients completed the longitudinal arm. Three generic questionnaires (Psychological General Well-Being Schedule [PGWBS], 36-item Short-Form [SF-36], EuroQoL [EQ-5D]) and the disease-specific acromegaly QoL questionnaire (AcroQoL) were applied. Quality of life assessment was performed 11.6±8.2 years following diagnosis and treatment of acromegaly. Patients with treated acromegaly had lower QoL scores compared with controls in all questionnaires with the exception of the PGWBS "Anxiety" subscale. The AcroQoL "Appearance" subscale and the "Physical Function" subscales of the remaining questionnaires were the most underscored domains. No difference in the total and subscale scores of all questionnaires was observed between baseline and follow-up, with the exception of the SF-36 "Physical Function," where a decline was found (58.5±24.7% vs 43.1±31.1%; P=.002). However, after adjusting for covariates, no significant change in any of the QoL scores was seen. Duration of IGF-1/GH control was positively correlated with QoL scores in most questionnaires at baseline, whereas use of GH lowering therapy at the time of QoL assessment was a negative predictive factor of QoL. Patients with biochemically controlled acromegaly demonstrate impaired QoL, which persists despite long-term disease control. This primarily consists of impaired physical function and secondly of impaired

  10. The Effect of the Exon-3-Deleted Growth Hormone Receptor on Pegvisomant-Treated Acromegaly: A Systematic Review and Meta-Analysis.

    Science.gov (United States)

    Franck, Sanne E; Broer, Linda; van der Lely, Aart Jan; Kamenicky, Peter; Bernabéu, Ignacio; Malchiodi, Elena; Delhanty, Patric J D; Rivadeneira, Fernando; Neggers, Sebastian J C M M

    2017-01-01

    The common exon 3 deletion polymorphism of the growth hormone receptor (d3-GHR) is associated with disease severity in acromegaly patients. The GHR antagonist pegvisomant (PEGV) is highly effective in treating severe acromegaly. Response to PEGV treatment seems to be influenced by d3-GHR and appears to be more responsive to PEGV, although available results remain conflicting. To assess the influence of d3-GHR on the responsiveness of acromegaly patients to PEGV by compiling the evidence derived from the largest available studies. A systematic review of the literature identified three published studies and one conference abstract. Acromegaly patients (n = 324, 49.7% d3-GHR carriers) were treated with either PEGV monotherapy or PEGV combined with long-acting somatostatin analogues and/or cabergoline. A meta-analysis of raw data from these studies was performed. No significant effect of the d3-GHR was observed while bringing insulin-like growth factor I (IGF-I) levels below the upper limit of normal with PEGV, which was defined as the lowest IGF-I level during PEGV treatment (mean difference: -2.3%; 95% CI: -6.5 to 1.8, p = 0.270). The PEGV dose required to achieve the lowest IGF-I levels was also not significantly influenced by individuals carrying d3-GHR (mean difference: 4.1 mg weekly; 95% CI: -5.1 to 13.2, p = 0.385). For both outcomes, separate analysis of PEGV monotherapy and combination treatment gave similar results. Our findings suggest that the d3-GHR polymorphism has no effect on biochemical disease control in acromegaly, as it is not of added value for either the prediction of PEGV responsiveness or the determination of the required PEGV dose. © 2016 The Author(s) Published by S. Karger AG, Basel.

  11. A Case of Acromegaly in which a Pituitary Gland Tumor was Reduced Significantly by Administering Octreotide Long Acting Release (LAR) and Could Be Removed Surgically.

    Science.gov (United States)

    Arao, Tadashi; Okada, Yosuke; Uemura, Fumi; Nishizawa, Shigeru; Tanaka, Yoshiya

    A 54-year-old woman was admitted to our hospital for detailed examination of acromegaly because she noticed bilateral hand and finger swelling at the age of 43 and plantar thickening, facial changes and unclear articulation at the age of 49. She had prominent brow ridges, mandibular protrusion, and enlargement of the hands, feet, nasal wings, lips and tongue. Her growth hormone (GH) level was 39.8 ng/ml, insulin-like growth factor-1 (IGF-1) level was 717 ng/ml, GH level was not suppressed (22.9 ng/ml) during a 75-g oral glucose tolerance test (OGTT). Radiography showed cauliflower-like enlargement of the distal phalanx of the fingers, thickening/enlargement of the plantar soft tissues, and increased antero-posterior diameter of the sella turcica. Magnetic resonance imaging showed a mass (21×17 mm) growing towards the right suprasellar region and invading the cavernous sinus. She was diagnosed with acromegaly based on the characteristic physical findings, GH excess, high IGF-1, lack of GH suppression during the 75-g OGTT, and the presence of a pituitary tumor. She was started on octreotide long acting release (Oct-LAR) 20 mg/4w for tumor shrinkage. After three doses, her GH and IGF-1 levels decreased to 2.19 ng/ml (1.69 during the 75-g OGTT) and 205 ng/ml, respectively, meeting cure criteria for acromegaly. In this case, a decrease in GH and IGF-1 levels, tumor shrinkage, and resolution of cavernous sinus invasion allowed the patient to undergo surgery with curative intent (the first-line treatment for acromegaly) without postoperative complications. Thus, preoperative Oct-LAR administration has the potential to improve treatment outcomes of acromegaly.

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

    Directory of Open Access Journals (Sweden)

    Marcelo Palmeira Rodrigues

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

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

    Directory of Open Access Journals (Sweden)

    Jose Hernan Martinez

    2011-01-01

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

  14. Hyperthyroidism and acromegaly due to a thyrotropin- and growth hormone-secreting pituitary tumor. Lack of hormonal response to bromocriptine.

    Science.gov (United States)

    Carlson, H E; Linfoot, J A; Braunstein, G D; Kovacs, K; Young, R T

    1983-05-01

    A 47-year-old woman with acromegaly and hyperthyroidism was found to have an inappropriately normal serum thyrotropin level (1.5 to 2.5 microU/ml) that responded poorly to thyrotropin-releasing hormone but showed partial responsiveness to changes in circulating thyroid hormones. Serum alpha-subunit levels were high-normal and showed a normal response to thyrotropin-releasing hormone. Growth hormone and thyrotropin hypersecretion persisted despite radiotherapy and bromocriptine treatment. Selective trans-sphenoidal removal of a pituitary adenoma led to normalization of both growth hormone and thyrotropin levels. Both thyrotropes and somatotropes were demonstrated in the adenoma by the immunoperoxidase technique and electron microscopy.

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

    Directory of Open Access Journals (Sweden)

    Mariângela Resende

    2012-02-01

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

  16. A multicenter, observational study of lanreotide depot/autogel (LAN) in patients with acromegaly in the United States: 2-year experience from the SODA registry.

    Science.gov (United States)

    Salvatori, Roberto; Gordon, Murray B; Woodmansee, Whitney W; Ioachimescu, Adriana G; Carver, Don W; Mirakhur, Beloo; Cox, David; Molitch, Mark E

    2017-12-01

    This analysis evaluates the 2-year effectiveness and safety of lanreotide depot/autogel (LAN), as well as treatment convenience and acromegaly symptom relief, from the Somatuline ® Depot for Acromegaly (SODA) registry, a post-marketing, open-label, observational, multicenter, United States registry study. Patients with acromegaly treated with LAN were eligible for enrollment. Demographics, LAN dose, extended dosing interval (EDI) (interval of injections ≥42 days), insulin-like growth factor 1 (IGF-1), growth hormone (GH), glycated hemoglobin, adverse events (AEs), injection convenience, and symptom data were collected. As of September 29, 2014, 241 patients were enrolled in SODA. IGF-1 levels below age- and gender-adjusted upper normal limit (ULN) were achieved in 71.2% at month (M) 12 and 74.4% at M24; GH ≤2.5 µg/L in 83.3% at M12 and 80.0% at M24; GH convenient. SODA indicates 2-year biochemical control with majority of patients achieving both IGF-1 < ULN and GH ≤2.5 µg/L. LAN was generally well tolerated with no new or unexpected safety signals reported during the observation period. clinicaltrials.gov Clinical Trial Identifier: NCT00686348.

  17. AMERICAN ASSOCIATION OF CLINICAL ENDOCRINOLOGISTS AND AMERICAN COLLEGE OF ENDOCRINOLOGY DISEASE STATE CLINICAL REVIEW: MANAGEMENT OF ACROMEGALY PATIENTS: WHAT IS THE ROLE OF PRE-OPERATIVE MEDICAL THERAPY?

    Science.gov (United States)

    Fleseriu, Maria; Hoffman, Andrew R; Katznelson, Laurence

    2015-06-01

    Acromegaly is a complex disease characterized by growth hormone (GH) excess originating in most cases from a pituitary tumor. The goals of treatment include removing the tumor or reducing tumor burden, normalizing GH secretion and insulin-like growth factor 1 levels, and preserving normal pituitary function if possible. Surgery by an experienced neurosurgeon is still considered first-line therapy, especially in cases with small tumors. In the last few decades, significant progress in the development of selective pharmacologic agents has greatly facilitated the management of active acromegaly, with agents such as somatostatin-receptor ligands (SRLs), GH-receptor antagonists, and dopamine agonists. In addition to adjuvant treatment, pre-operative medical therapy and primary therapy in de novo patients are increasingly employed. A United States National Library of Medicine PubMed search (through July 2014) was conducted for the following terms: acromegaly, pre-operative medical therapy, somatostatin-receptor ligands, and somatostatin analogs. Articles not in English and those not in peer-reviewed journals were excluded. In reviewing pertinent articles, focus was placed on biochemical and other postoperative outcomes of medical therapy. An analysis of the full effect of pre-operative use of SRLs on surgical outcomes (remission rates and peri-operative complications) is limited by heterogeneity of methodology, low overall surgical cure rates, and different study designs. The assumption that SRL use prior to surgery reduces peri-operative surgical risk has yet to be proven. A variable degree of tumor shrinkage with preoperative SRLs is observed. Likewise, SRL treatment 3 months before surgery may improve surgical remission rates in the short term; however, positive results do not persist in the long term. We consider that medical therapy before surgery could play a role in carefully selected patients, but treatment should be individualized. Primary medical therapy with a

  18. Evaluation of blood neutrophil to lymphocyte and platelet to lymphocyte ratios according to plasma glucose status and serum insulin-like growth factor 1 levels in patients with acromegaly.

    Science.gov (United States)

    Üçler, R; Aslan, M; Atmaca, M; Alay, M; Ademoğlu, E N; Gülşen, I

    2016-06-01

    Cardiovascular, respiratory, and cerebrovascular diseases and malignancies are responsible for morbidity and mortality in acromegaly. Also these diseases are associated with chronic inflammation. The neutrophil to lymphocyte ratio (NLR) and platelet to lymphocyte ratio (PLR) are currently gaining interest as new markers of inflammation. Moreover, increased morbidity and mortality are positively correlated with the presence of diabetes and levels of insulin-like growth factor 1 (IGF-1) in acromegaly. The objective of the present study was to investigate the relationship between these markers and acromegaly according to plasma glucose status and serum IGF-1 levels. We retrospectively analyzed data from 61 acromegaly patients who were in a newly diagnosed period (35 male, 26 female; mean age 38.13 ± 13.98). Patients with normal plasma glucose (n = 27), impaired fasting glucose (n = 18), and diabetes mellitus (n = 16) were categorized into three different groups. NLR and PLR were compared between the study groups and were evaluated according to IGF-1 levels. There were no statistically significant differences in NLR and PLR measurements among the study groups (p > 0.05). However, there were significant positive correlations between NLR and IGF-1 levels and between PLR and IGF-1 levels when all patients were evaluated (r = 0.334, p = 0.011 and r = 0.277, p = 0.035, respectively). This is the first report studying the relationship of NLR and PLR with glucose status and IGF-1 levels in acromegaly patients. Our study results suggest that subclinical inflammation may play a role in increased incidence of mortality and morbidity, which depends on uncontrolled IGF-1 levels in patients with acromegaly. © The Author(s) 2015.

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

    DEFF Research Database (Denmark)

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

    2007-01-01

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

  20. Treatment of acromegaly patients at the Federal University of Triângulo Mineiro (UFTM: Experience Report

    Directory of Open Access Journals (Sweden)

    Maria de Fátima Borges

    Full Text Available OBJECTIVE: To evaluate the effectiveness of the treatment of acromegaly patients at the Federal University of Triangulo Mineiro. METHODS: Cross-sectional and retrospective study of thirty cases treated over a period of two decades. RESULTS: 17 men (56.7% aged 14-67 years and 13 women aged 14-86 years were analyzed. Twenty-one patients underwent transphenoidal surgery, whichwas associated with somatostatin receptor ligands in 11 patients (39.3%, somatostatin receptor ligands + radiotherapyin 5 patients (17.8%, radiotherapy in 3 patients (10.7%, and radiotherapy + somatostatin receptorligands + cabergoline in 1 patient (3.6%. Additionally, 2 patients underwent radiotherapy and surgeryalone. Six patients received somatostatin receptor ligands before surgery, and 2 were not treated due to refusal and death. Nine patients have died, and 20 are being followed; 13 (65% have growth hormonelevels o1 ng/mL, and 11 have normal insulin-like growth factor 1 levels. CONCLUSION: The current treatment options enable patients seen in regional reference centers to achieve strict control parameters, which allows them to be treated close to their homes.

  1. An orphan G-protein-coupled receptor causes human gigantism and/or acromegaly: Molecular biology and clinical correlations.

    Science.gov (United States)

    Trivellin, Giampaolo; Hernández-Ramírez, Laura C; Swan, Jeremy; Stratakis, Constantine A

    2018-04-01

    X-linked acrogigantism (X-LAG) is a recently described form of familial or sporadic pituitary gigantism characterized by very early onset GH and IGF-1 excess, accelerated growth velocity, gigantism and/or acromegaloid features. Germline or somatic microduplications of the Xq26.3 chromosomal region, invariably involving the GPR101 gene, constitute the genetic defect leading to X-LAG. GPR101 encodes a class A G protein-coupled receptor that activates the 3',5'-cyclic adenosine monophosphate signaling pathway. Highly expressed in the central nervous system, the main physiological function and ligand of GPR101 remain unknown, but it seems to play a role in the normal development of the GHRH-GH axis. Early recognition of X-LAG cases is imperative because these patients require clinical management that differs from that of other patients with acromegaly or gigantism. Medical treatment with pegvisomant seems to be the best approach, since X-LAG tumors are resistant to the treatment with somatostatin analogues and dopamine agonists; surgical cure requires near-total hypophysectomy. Currently, the efforts of our research focus on the identification of GPR101 ligands; in addition, the long-term follow-up of X-LAG patients is of extreme interest as this is expected to lead to better understanding of GPR101 effects on human pathophysiology. Copyright © 2018. Published by Elsevier Ltd.

  2. Treatment of acromegaly patients at the Federal University of Triângulo Mineiro (UFTM): Experience Report

    Science.gov (United States)

    de Fátima Borges, Maria; Lara, Beatriz Hallal Jorge; Tomé, Janaíne Machado; de Araújo, Leopoldo Prezia; Bugiga, Flávio Cesar Lucas; Sousa, Júlio Cláudio; Soares, Jacqueline Mendes Fonseca; Dezena, Roberto Alexandre; Ferreira, Beatriz Pires

    2017-01-01

    OBJECTIVE: To evaluate the effectiveness of the treatment of acromegaly patients at the Federal University of Triangulo Mineiro. METHODS: Cross-sectional and retrospective study of thirty cases treated over a period of two decades. RESULTS: 17 men (56.7%) aged 14-67 years and 13 women aged 14-86 years were analyzed. Twenty-one patients underwent transphenoidal surgery, whichwas associated with somatostatin receptor ligands in 11 patients (39.3%), somatostatin receptor ligands + radiotherapyin 5 patients (17.8%), radiotherapy in 3 patients (10.7%), and radiotherapy + somatostatin receptorligands + cabergoline in 1 patient (3.6%). Additionally, 2 patients underwent radiotherapy and surgeryalone. Six patients received somatostatin receptor ligands before surgery, and 2 were not treated due to refusal and death. Nine patients have died, and 20 are being followed; 13 (65%) have growth hormonelevels o1 ng/mL, and 11 have normal insulin-like growth factor 1 levels. CONCLUSION: The current treatment options enable patients seen in regional reference centers to achieve strict control parameters, which allows them to be treated close to their homes. PMID:28492721

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

    Directory of Open Access Journals (Sweden)

    Jan-Shun Chang

    2016-06-01

    Full Text Available 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.

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

    Directory of Open Access Journals (Sweden)

    Connock Martin J

    2009-10-01

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

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

    DEFF Research Database (Denmark)

    Parkinson, C; Kassem, M; Heickendorff, Lene

    2003-01-01

    of bone and soft tissue turnover, as well as levels of PTH and vitamin D metabolites, in 16 patients (nine males; median age, 52 yr; range, 28-78 yr) with active acromegaly (serum IGF-I at least 30% above upper limit of an age-related reference range). Serum procollagen III amino-terminal propeptide...... (PIIINP) and type I procollagen amino-terminal propeptide, osteocalcin (OC), bone-related alkaline phosphatase, C-terminal cross-linked telopeptide of type I collagen (CTx), albumin-corrected calcium, intact PTH, 25-hydroxy vitamin D, 1,25-dihydroxy vitamin D [1,25-(OH)(2) vit D], urinary type 1 collagen...... (CTx and urinary type 1 collagen cross-linked N-telopeptide/creatinine ratio). 1,25-(OH)(2) vit D decreased and intact PTH increased significantly, but 25-hydroxy vitamin D was unaffected. A significant decline in calculated calcium clearance was observed. The decrease in serum IGF-I correlated...

  6. Efficacy and safety of long-acting pasireotide in Japanese patients with acromegaly or pituitary gigantism: results from a multicenter, open-label, randomized, phase 2 study.

    Science.gov (United States)

    Tahara, Shigeyuki; Murakami, Mami; Kaneko, Tomomi; Shimatsu, Akira

    2017-07-28

    A multicenter, open-label, phase 2 study was conducted to investigate the efficacy and safety of long-acting pasireotide formulation in Japanese patients with acromegaly or pituitary gigantism. Medically naïve or inadequately controlled patients (on somatostatin analogues or dopamine agonists) were included. Primary end point was the proportion of all patients who achieved biochemical control (mean growth hormone [GH] levelsacromegaly, n=32; pituitary gigantism, n=1) were enrolled and randomized 1:1:1 to receive open-label pasireotide 20mg, 40mg, or 60mg. The median age was 52 years (range, 31-79) and 20 patients were males. At month 3, 18.2% of patients (6/33; 90% confidence interval: 8.2%, 32.8%) had biochemical control (21.2% [7/33] when including a patient with mean GHacromegaly or pituitary gigantism.

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

    Science.gov (United States)

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

    2016-12-01

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

  8. The growth hormone (GH) response to GH-releasing peptide (His-DTrp-Ala-Trp-DPhe-Lys-NH2), GH-releasing hormone, and thyrotropin-releasing hormone in acromegaly.

    Science.gov (United States)

    Alster, D K; Bowers, C Y; Jaffe, C A; Ho, P J; Barkan, A L

    1993-09-01

    In patients with acromegaly, GH-producing pituitary tumors release GH in response to specific stimuli such as GH-releasing hormone (GHRH) and are also responsive to a variety of nonspecific stimuli, such as TRH or GnRH, and may exhibit paradoxical responses to glucose and dopamine. In healthy humans, the synthetic peptide GH-releasing peptide (GHRP) (His-D-Trp-Ala-Trp-D-Phe-Lys-NH2) releases GH by a putative mechanism of action that is independent of GHRH. How these tumors respond to GHRP is not well characterized. We studied the GH responses to GHRH, GHRP, and TRH stimulation in 11 patients with active acromegaly. The peak GH responses to GHRP and GHRH were not correlated (r = 0.57; P = 0.066). In contrast, the peak GH responses to GHRP and TRH were highly correlated (r = 0.95; P < 0.001). In conclusion, in patients with acromegaly, the GH response to GHRP is qualitatively normal and does not appear to depend on GHRH.

  9. Acromegaly Presenting with Hemiplegia

    African Journals Online (AJOL)

    1974-04-06

    Apr 6, 1974 ... noticed progressive enlargement of her hands and feet during the previous 9 years and now needed shoes which ... Lateral radiograph of the foot and heel, demon- strating increased heel pad thickness. Heel pad ... film with palms flat on a cassette. The greatest diameter in millimetres of the medial ...

  10. Delayed Diagnosis of Acromegaly in the Context of Post-Traumatic Stress Disorder due to Symptoms Mimicking Known Psychotropic Medication Side Effects.

    Science.gov (United States)

    Portier, Ray-Bernard; Afarin, Afshin; Pope, Sara

    2017-07-01

    Acromegaly is caused by elevated secretion of human growth hormone, which is frequently because of intracranial tumors. This diagnosis is fairly uncommon with an incidence of 3 to 4 cases per million patients per year. We are presenting a case of acromegaly diagnosed in an active duty Chief Petty Officer. A 38-year-old male Chief Petty Officer with no previous mental health diagnosis experienced post-traumatic stress disorder (PTSD)-like symptoms in early 2012 after deploying to Iraq and Afghanistan from 2010 to 2011. Initially he self-managed his symptoms, but in July 2012 he required a reduction mammoplasty because of gynecomastia. The metabolic workup revealed elevated prolactin, but this was not further investigated. His recovery from anesthesia was complicated by intensified PTSD-like symptoms, which continued to worsen after the surgery. On self-referral to mental health, he was diagnosed with PTSD and managed for 6 months with cognitive behavioral therapy. Because of persistent and worsening symptoms, his therapy was augmented to include continued cognitive behavioral therapy, alpha-blockers, antidepressants, antihistamines, and sleep aids. Because of night sweats, the selective serotonin reuptake inhibitors doses were modified. Night sweats persisted, and the patient was re-evaluated for other potential etiologies. On evaluation, the patient endorsed a history of obstructive sleep apnea, cervicalgia, visual changes, depressed mood, as well as multiple physical symptoms including coarsened facial features, large hands/feet, and increased interdental distance. On laboratory analysis, insulin-like growth factor 1 was noted to be 3 times the upper limit of normal, and a prolactin level was five times the upper limit of normal. A brain magnetic resonance imaging revealed a cystic pituitary lesion with suprasellar extension, compression of the infundibulum without invasion of the cavernous sinus, or displacement of the optic chiasm. Based on clinical history

  11. Loss of heterozygosity on chromosome 11q13 in two families with acromegaly/gigantism is independent of mutations of the multiple endocrine neoplasia type I gene.

    Science.gov (United States)

    Gadelha, M R; Prezant, T R; Une, K N; Glick, R P; Moskal, S F; Vaisman, M; Melmed, S; Kineman, R D; Frohman, L A

    1999-01-01

    Familial acromegaly/gigantism occurring in the absence of multiple endocrine neoplasia type I (MEN-1) or the Carney complex has been reported in 18 families since the biochemical diagnosis of GH excess became available, and the genetic defect is unknown. In the present study we examined 2 unrelated families with isolated acromegaly/gigantism. In family A, 3 of 4 siblings were affected, with ages at diagnosis of 19, 21, and 23 yr. In family B, 5 of 13 siblings exhibited the phenotype and were diagnosed at 13, 15, 17, 17, and 24 yr of age. All 8 affected patients had elevated basal GH levels associated with high insulin-like growth factor I levels and/or nonsuppressible serum GH levels during an oral glucose tolerance test. GHRH levels were normal in affected members of family A. An invasive macroadenoma was found in 6 subjects, and a microadenoma was found in 1 subject from family B. The sequence of the GHRH receptor complementary DNA in 1 tumor from family A was normal. There was no history of consanguinity in either family, and the past medical history and laboratory results excluded MEN-1 and the Carney complex in all affected and unaffected screened subjects. Five of 8 subjects have undergone pituitary surgery to date, and paraffin-embedded pituitary blocks were available for analysis. Loss of heterozygosity on chromosome 11q13 was studied by comparing microsatellite polymorphisms of leukocyte and tumor DNA using PYGM (centromeric) and D11S527 (telomeric), markers closely linked to the MEN-1 tumor suppressor gene. All tumors exhibited a loss of heterozygosity at both markers. Sequencing of the MEN-1 gene revealed no germline mutations in either family, nor was a somatic mutation found in tumor DNA from one subject in family A. The integrity of the MEN-1 gene in this subject was further supported by demonstration of the presence of MEN-1 messenger ribonucleic acid, as assessed by RT-PCR. These data indicate that loss of heterozygosity in these affected family

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

    Science.gov (United States)

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

    2015-01-01

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

  13. 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

    Directory of Open Access Journals (Sweden)

    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.

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

    Directory of Open Access Journals (Sweden)

    Loraine Ledón Llanes

    2011-08-01

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

  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

    International Nuclear Information System (INIS)

    Bostroem, Jan Patrick; Kinfe, Thomas; Pintea, Bogdan; Meyer, Almuth; Gerlach, Ruediger; Surber, Gunnar; Hamm, Klaus; Lammering, Guido

    2015-01-01

    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.) [de

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

    Energy Technology Data Exchange (ETDEWEB)

    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

  17. Effects of conventional high-voltage irradiation and proton irradiation as adjuncts to surgical measures in acromegaly with particular attention being given to the further course of illness with and without drug treatment

    International Nuclear Information System (INIS)

    Lutz, B.S.

    1986-01-01

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

  18. Patients' experience of nursing with Acromegaly

    DEFF Research Database (Denmark)

    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....

  19. New Insights into Medical Treatment of Acromegaly

    NARCIS (Netherlands)

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

    2011-01-01

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

  20. New Insights into Medical Treatment of Acromegaly

    OpenAIRE

    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...

  1. An abnormal carbohydrate tolerance in acromegaly

    International Nuclear Information System (INIS)

    Qi Jinwu

    1988-01-01

    An abnormal secretion of plasma human growth hormore (hGH) and insulin in 67 acromegalic patients had been previously treated by external pituitary radiation were studied. All subjects, following an overnight fast, a standard 100 g oral glucose tolerance test, were performed and venous blood samples were taken at 0, 30, 60, 120 and 180 min. They were measured for blood glucose, plasma insulin and hGH. The results of this study have shown that, of the 67 subjects, 23 cases had an abnormal glucose tolerance(34.32%). Diabetes was detected in 17 cases (23.37%) and 6 patients had decreased glucose tolerance(8.69%). In all, hGH levels were consistantly above 5 ng/ml and were not suppressed after an oral glucose load. In these patients, however, about one-third had abnormal glucose tolerance. Low plasma insulin response to glucose and that of the releasing were evident in them than the normal glucose tolerance and a healthy control group. In addition, the mechanism of the abnormal secretion of hGH and insulin were disscussed

  2. Hypopituitarism following pituitary irradiation for acromegaly

    Energy Technology Data Exchange (ETDEWEB)

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

    1978-01-01

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

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

    Energy Technology Data Exchange (ETDEWEB)

    Lutz, B.S.

    1986-09-03

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

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

    Energy Technology Data Exchange (ETDEWEB)

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

    1997-06-01

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

  5. Acromegaly resolution after traumatic brain injury: a case report

    OpenAIRE

    Cob, Alejandro

    2014-01-01

    Introduction Anterior hypopituitarism is a common complication of head trauma, with a prevalence of 30% to 70% among long-term survivors. This is a much higher frequency than previously thought and suggests that most cases of post-traumatic hypopituitarism remain undiagnosed and untreated. Symptoms of hypopituitarism are very unspecific and very similar to those in traumatic brain injury patients in general, which makes hypopituitarism difficult to diagnose. The factors that predict the likel...

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

    International Nuclear Information System (INIS)

    Rosenstock, J.; Doyle, F.H.; Mashiter, K.; Joplin, G.F.

    1982-01-01

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

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

    Energy Technology Data Exchange (ETDEWEB)

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

    1982-01-01

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

  8. Skin morphological changes in growth hormone deficiency and acromegaly

    DEFF Research Database (Denmark)

    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....

  9. Changes in bone metabolism during treatment of acromegaly

    NARCIS (Netherlands)

    Bijlsma, J. W.; Nortier, J. W.; Duursma, S. A.; Croughs, R. J.; Bosch, R.; Thijssen, J. H.

    1983-01-01

    Bone metabolism was studied in 17 acromegalic patients, who responded to either medical treatment with bromocriptine (12 patients), or to transsphenoidal surgery (5 patients). Parameters of bone turnover decreased, e.g. serum acid phosphatase (9.2 +/- 0.7 vs 8.1 +/- 0.6 U/l, P less than 0.05) and

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

    Czech Academy of Sciences Publication Activity Database

    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

  11. Glucosetolerantie, insulinereserve en micro-angiopathie. Een onderzoek bij prediabetes, acromegalie en hypertriglyceridemie

    NARCIS (Netherlands)

    Vries, Ouwe de

    1975-01-01

    In hoofdstuk I wordt een literatuuroverzicht gegeven van de kennis van de insuline-afgifte, in nuchtere toestand en na stimulatie, bij controlepersonen, bij patienten met diabetes mellitus en bij genetisch belaste personen rret een normale glucosetolerantie. ... Zie: Samenvatting

  12. Long-term follow-up of patients with surgical intractable acromegaly after linear accelerator radiosurgery

    Directory of Open Access Journals (Sweden)

    Jiun-Lin Yan

    2013-07-01

    Conclusion: In comparison to other radiosurgery modalities, LINAC radiosurgery also provides a satisfactory outcome. SRS has maximum effect over the first 2 years and stabilizes after 7.5 years. Moreover, SRS elicits long-term biochemical effects and requires longer follow-up for better biochemical remission.

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

    International Nuclear Information System (INIS)

    Reed, P.I.; Joplin, G.F.; Speirs, C.J.; Morrison, R.; Aber, V.

    1990-01-01

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

  14. Long-term effect of 90Y pituitary implantation in acromegaly

    International Nuclear Information System (INIS)

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

    1987-01-01

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

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

    Directory of Open Access Journals (Sweden)

    Ji Young Park

    2014-12-01

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

  16. Stubborn hiccups as a sign of massive apoplexy in a naive acromegaly patient with pituitary macroadenoma

    Directory of Open Access Journals (Sweden)

    Gulay Simsek Bagir

    2017-05-01

    Full Text Available Pituitary apoplexy (PA may very rarely present with hiccups. A 32-year-old man with classical acromegaloid features was admitted with headache, nausea, vomiting and stubborn hiccups. Pituitary magnetic resonance imaging (MRI demonstrated apoplexy of a macroadenoma with suprasellar extension abutting the optic chiasm. Plasma growth hormone (GH levels exhibited suppression (below <1 ng/mL at all time points during GH suppression test with 75 g oral glucose. After treatment with corticosteroid agents, he underwent transsphenoidal pituitary surgery and hiccups disappeared postoperatively. The GH secretion potential of the tumor was clearly demonstrated immunohistochemically. We conclude that stubborn hiccups in a patient with a pituitary macroadenoma may be a sign of massive apoplexy that may result in hormonal remission.

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

    Energy Technology Data Exchange (ETDEWEB)

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

    1987-01-01

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

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

    International Nuclear Information System (INIS)

    Ciccarelli, E.; Corsello, S.M.; Besser, G.M.; Wass, J.A.H.; Plowman, P.N.; Jones, A.E.; Touzel, R.; Rees, L.H.

    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)

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

    Energy Technology Data Exchange (ETDEWEB)

    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).

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

    Directory of Open Access Journals (Sweden)

    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.

  1. Preliminary studies of plasma growth hormone releasing activity during medical therapy of acromegaly

    International Nuclear Information System (INIS)

    Hagen, T.C.; Lawrence, A.M.; Kirsteins, L.

    1978-01-01

    The in vitro growth hormone releasing activity of plasma obtained from six acromegalic subjects was measured before and during therapy. In five subjects, plasmas were obtained before and during successful medical therapy with medroxyprogesterone acetate (MPA). The sixth subject was sampled before and after transphenoidal Sr 90 -induced hypopituitarism. All subjects had a decrement in fasting growth hormone levels with respective therapies (29-88%). The in vitro growth hormone released from Rhesus monkey anterior pituitaries was assessed after incubating one lateral half in control plasma (pre-therapy) and the contralateral pituitary half in plasma obtained during or after therapy. Studies with plasmas obtained from the five patients successfully treated with MPA showed a decrease in growth hormone releasing activity during therapy in all (18-57%). Plasma obtained after Sr 90 pituitary ablation in the sixth subject had 35% more growth hormone releasing activity than obtained before therapy. These results suggest that active acromegalics who respond to MPA with significantly lowered growth hormone levels may actually achieve this response because of a decrease in growth hormone releasing factor measured peripherally. The opposite response in one acromegalic subject, following Sr 90 pituitary ablation and hypopituitarism, suggests that growth hormone releasing factor secretion may increase when growth hormone levels are lowered by ablative therapy. (orig.) [de

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

    Energy Technology Data Exchange (ETDEWEB)

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

    1990-01-01

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

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

    LENUS (Irish Health Repository)

    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.

  4. Low incidence of adrenal insufficiency after transsphenoidal surgery in patients with acromegaly: a long-term follow-up study

    NARCIS (Netherlands)

    Burgers, Anne Marij G.; Kokshoorn, Nieke E.; Pereira, Alberto M.; Roelfsema, Ferdinand; Smit, Johannes W. A.; Biermasz, Nienke R.; Romijn, Johannes A.

    2011-01-01

    The long-term prevalence of adrenal insufficiency after transsphenoidal surgery for GH-secreting pituitary adenomas is unknown. However, recently a single study reported a high prevalence of adrenal insufficiency in acromegalic patients after surgical and/or medical treatment without postoperative

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

    DEFF Research Database (Denmark)

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

    2014-01-01

    on GH. The safety population included 164 adults with acroGHD and 2469 with NFPA, all GH-replaced. Both acroGHD and NFPA were compared with several cohorts from the general population (including the World Health Organization Global Burden of Disease). OUTCOME MEASURES: Outcome measures included quality...

  6. Reduction of pituitary-tumour size in patients with prolactinomas and acromegaly treated with bromocriptine with or without radiotherapy

    International Nuclear Information System (INIS)

    Wass, J.A.H.; Moult, P.J.A.; Thorner, M.O.; Dacie, J.E.; Charlesworth, M.; Jones, A.E.; Besser, G.M.

    1979-01-01

    69 patients with prolactin-secreting or growth-hormone-secreting pituitary tumours were treated with bromocriptine with or without pituitary irradiation and followed up for 6 months to 6 1/2 years. Of 26 patients with prolactinomas, 11 had external pituitary irradiation in addition to bromocriptine. There was evidence of shrinkage of the pituitary tumour (either a reduction in fossa size or loss of visual-field defects) in 6 of these patients (23%), 3 of whom had been treated with bromocriptine alone. Of 43 acromegalic patients, 30 received external pituitary irradiation. 8 (19%) showed evidence of shrinkage of the pituitary tumour, including 2 who had received no radiotherapy. 1 patient treated with bromocriptine alone showed striking reduction in the size of his suprasella extension, as assessed by serial computed-tomography scans over 11 months. At the same time his visual-field defects resolved and his deficient corticotrophin and thyrotrophin reserves returned to normal. Bromocriptine can reduce the size of both prolactin-secreting and growth-hormone-secreting pituitary tumours, and this is of potential importance in their management. (author)

  7. Serum concentrations of somatomedins and growth hormone in relation to bone metabolism in acromegaly and thyroid dysfunction

    NARCIS (Netherlands)

    Bijlsma, J. W.; Duursma, S. A.

    1986-01-01

    Many hormones are involved in the complex process of formation and resorption of bone. However, only somatomedin is found to directly stimulate cell replication and collagen synthesis in bone. This study was undertaken to examine a possible regulatory role of somatomedin in mediating the effects of

  8. How are growth hormone and insulin-like growth factor-1 reported as markers for drug effectiveness in clinical acromegaly research? A comprehensive methodologic review

    NARCIS (Netherlands)

    Van Esdonk, Michiel J.; Van Zutphen, Eline J. M.; Roelfsema, Ferdinand; Pereira, Alberto M.; Van Der Graaf, Piet H.; Biermasz, Nienke R.; Stevens, Jasper; Burggraaf, Jacobus

    2018-01-01

    Objective. In rare disease research, most randomized prospective clinical trials can only use limited number of patients and are comprised of highly heterogeneous populations. Therefore, it is crucial to report the results in such a manner that it allows for comparison of treatment effectiveness and

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

    Science.gov (United States)

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

    2010-12-01

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

  10. Disease: H01483 [KEGG MEDICUS

    Lifescience Database Archive (English)

    Full Text Available atin analog responsiveness in acromegaly. ... JOURNAL ... J Clin Endocrinol Metab 90:6290-5 (2005) DOI:10.1210/j...gland: New consensus in acromegaly: criteria for cure and control. ... JOURNAL ... Nat Rev Endocrinol 6:480-1 (2...c.2005-0998 ... PMID:20720589 (marker) ... AUTHORS ... Biermasz N ... TITLE ... Pituitary

  11. Ambulatory Care Data Base (ACDB) Data Dictionary Sequential Files of Phase 2.

    Science.gov (United States)

    1992-04-01

    ASTHMA, BRONCHODILATOR DEP 49397 ASTHMA, STERIOD DEP 49398 ASTHMA, EXERCISE INDUCED 49399 ASTHMA, OCCUPATIONAL 494 BRONCHI ECTAS IS 495 ALVEOLITIS...ACROMEGALY 25300 ACROMEGALY & GIGANTISM 2531 PITUITARY HYPERFUNCTION, ANTERIOR, OTH & UNSP 25311 HYPERPROLACTINEMIA ANT PIT HYPERFUNCT 25312 AMENORRHEA ...DISORDER 32790 INTOXICATION, SUBS, UNSPEC 32791 WITHDRAWAL, SUBS, UNSPEC 32792 DELERIUM, SUBS UNSPEC 32793 DEMENTIA, DRUG INDUCED , SUBS UNSPEC 32794

  12. Ambulatory Care Data Base (ACDB) Data Dictionary Sequential Files of Phase 1

    Science.gov (United States)

    1989-11-01

    BRONCHODILATOR DEP 49397 ASTHMA, STERIOD DEP 49398 ASTHMA, EXERCISE INDUCED 49399 ASTHMA, OCCUPATIONAL 4914 BRONCHIECTASIS 495 ALVEOLITIS, ALLERGIC...ACROMEGALY 25300 ACROMEGALY & GIGANTISM 2531 PITUITARY HYPERFUNCTION, ANTERIOR, OTH & UNSP 25311 RYPERPROLACTINEMIA ANT PIT HYPERFUNCT 25312 AMENORRHEA ...UNSPEC 32792 DELERIUM, SUBS UNSPEC 32793 DEMENTIA, DRUG INDUCED , SUBS UNSPEC 32794 AMNESTIC DISORDER, DRUG INDUCED , SUBS, UNSPEC 32795 DELUSIONAL

  13. Disease: H01864 [KEGG MEDICUS

    Lifescience Database Archive (English)

    Full Text Available (GH) causes acromegaly (H01483) and pituitary gigantism (H01618). They have the same pathogenetic mechanism...nd IGF-I levels ... See also H01483 Acromegaly and H01618 Pituitary gigantism. ICD-1...0: E22.0 MeSH: D000172 D005877 OMIM: 102200 300943 PMID:22584702 ... AUTHORS ... Herder WW ... TITLE ... Familial gigantism

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

    NARCIS (Netherlands)

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

    1993-01-01

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

  15. Disease: H01618 [KEGG MEDICUS

    Lifescience Database Archive (English)

    Full Text Available probably causes gigantism and acromegaly. Therapeutic modalities for the treatme...atakis CA ... TITLE ... Gigantism and acromegaly due to Xq26 microduplications and GPR101 mutation. ... JOURNAL ... N Engl J Med 371:2363-74 (2014) DOI:10.1056/NEJMoa1408028 ...

  16. Sweat secretion rates in growth hormone disorders

    DEFF Research Database (Denmark)

    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....

  17. Growth hormone and insulin-like growth factor 1 affect the severity of Graves' disease.

    Science.gov (United States)

    Di Cerbo, Alfredo; Pezzuto, Federica; Di Cerbo, Alessandro

    2017-01-01

    Graves' disease, the most common form of hyperthyroidism in iodine-replete countries, is associated with the presence of immunoglobulins G (IgGs) that are responsible for thyroid growth and hyperfunction. In this article, we report the unusual case of a patient with acromegaly and a severe form of Graves' disease. Here, we address the issue concerning the role of growth hormone (GH) and insulin-like growth factor 1 (IGF1) in influencing thyroid function. Severity of Graves' disease is exacerbated by coexistent acromegaly and both activity indexes and symptoms and signs of Graves' disease improve after the surgical remission of acromegaly. We also discuss by which signaling pathways GH and IGF1 may play an integrating role in regulating the function of the immune system in Graves' disease and synergize the stimulatory activity of Graves' IgGs. Clinical observations have demonstrated an increased prevalence of euthyroid and hyperthyroid goiters in patients with acromegaly.The coexistence of acromegaly and Graves' disease is a very unusual event, the prevalence being Graves' disease associated with acromegaly and show that surgical remission of acromegaly leads to a better control of symptoms of Graves' disease.

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

    Science.gov (United States)

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

    2005-09-01

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

  19. Unusual AIP mutation and phenocopy in the family of a young patient with acromegalic gigantism

    OpenAIRE

    Syed Ali Imran; Khaled A Aldahmani; Lynette Penney; Sidney E Croul; David B Clarke; Donato Iacovazzo; Márta Korbonits

    2018-01-01

    Summary Early-onset acromegaly causing gigantism is often associated with aryl-hydrocarbon-interacting receptor protein (AIP) mutation, especially if there is a positive family history. A15y male presented with tiredness and visual problems. He was 201 cm tall with a span of 217 cm. He had typical facial features of acromegaly, elevated IGF-1, secondary hypogonadism and a large macroadenoma. His paternal aunt had a history of acromegaly presenting at the age of 35 years. Following transspheno...

  20. Growth hormone suppression test

    Science.gov (United States)

    ... Acromegaly - blood test; Gigantism - blood test Images Blood test References Kaiser U, Ho KKY. Pituitary physiology and diagnostic evaluation. In: Melmed S, Polonsky KS, Larsen PR, Kronenberg HM, eds. Williams Textbook of Endocrinology . 13th ed. Philadelphia, PA: Elsevier; ...

  1. South African Medical Journal - Vol 45, No 9 (1971)

    African Journals Online (AJOL)

    Hyperglycaemic non-ketotic coma following surgically treated acromegaly in a non-diabetic · EMAIL FREE FULL TEXT EMAIL FREE FULL TEXT DOWNLOAD FULL TEXT DOWNLOAD FULL TEXT. W.P.U. Jackson, B.L. Pimpstone ...

  2. Growth hormone test

    Science.gov (United States)

    ... is called acromegaly . In children it is called gigantism . Too little growth hormone can cause a slow ... growth due to excess GH during childhood, called gigantism. (A special test is done to confirm this ...

  3. Genetics Home Reference: familial isolated pituitary adenoma

    Science.gov (United States)

    ... children or adolescents can lead to increased height (gigantism), because the long bones of their arms and ... Information & Resources MedlinePlus (6 links) Encyclopedia: Acromegaly Encyclopedia: Gigantism Encyclopedia: Pituitary Tumor Encyclopedia: Prolactinoma Health Topic: Endocrine ...

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

    DEFF Research Database (Denmark)

    Andreassen, Mikkel; Faber, Jens; Vestergaard, Henrik

    2007-01-01

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

  5. Management of endocrine disease: GH excess: diagnosis and medical therapy

    DEFF Research Database (Denmark)

    Andersen, Marianne

    2014-01-01

    Acromegaly is predominantly caused by a pituitary adenoma, which secretes an excess of GH resulting in increased IGF-I levels. Most of the GH assays used currently measure only the 22 kDa form of GH. In theory, the diagnostic sensitivity may be lower compared to the previous assays, which used...... polyclonal antibodies. Many GH-secreting adenomas are plurihormonal and may co-secrete prolactin, TSH and α-subunit. Hyperprolactinemia is found in 30-40% of patients with acromegaly and hyperprolactinemia may occasionally be diagnosed before acromegaly is apparent.Although trans-sphenoidal surgery of a GH......-secreting adenoma remains the first treatment at most centres, the role of somatostatin analogues, octreotide LAR and lanreotide Autogel, as primary therapy is still the subject of some debate. While normalization of GH and IGF-I levels is the main objective in all patients with acromegaly, GH and IGF-I levels may...

  6. McCune-Albright syndrome

    Science.gov (United States)

    ... bones in the face Gigantism Irregular, large patchy cafe-au-lait spots , especially on the back ... skull Abnormal heart rhythms ( arrhythmias ) Acromegaly Gigantism Large cafe-au-lait spots on the skin Liver disease, ...

  7. Pituitary tumors containing cholecystokinin

    DEFF Research Database (Denmark)

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

    1987-01-01

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

  8. AIP mutations and gigantism.

    Science.gov (United States)

    Rostomyan, Liliya; Potorac, Iulia; Beckers, Pablo; Daly, Adrian F; Beckers, Albert

    2017-06-01

    AIP mutations are rare in sporadic acromegaly but they are seen at a higher frequency among certain specific populations of pituitary adenoma patients (pituitary gigantism cases, familial isolated pituitary adenoma (FIPA) kindreds, and patients with macroadenomas who are diagnosed ≤30 years). AIP mutations are most prevalent in patients with pituitary gigantism (29% of this group were found to have mutations in AIP gene). These data support targeted genetic screening for AIP mutations/deletions in these groups of pituitary adenoma patients. Earlier diagnosis of AIP-related acromegaly-gigantism cases enables timely clinical evaluation and treatment, thereby improving outcomes in terms of excessive linear growth and acromegaly comorbidities. Copyright © 2017 Elsevier Masson SAS. All rights reserved.

  9. Unusual AIP mutation and phenocopy in the family of a young patient with acromegalic gigantism

    Directory of Open Access Journals (Sweden)

    Syed Ali Imran

    2018-01-01

    Full Text Available Early-onset acromegaly causing gigantism is often associated with aryl-hydrocarbon-interacting receptor protein (AIP mutation, especially if there is a positive family history. A15y male presented with tiredness and visual problems. He was 201 cm tall with a span of 217 cm. He had typical facial features of acromegaly, elevated IGF-1, secondary hypogonadism and a large macroadenoma. His paternal aunt had a history of acromegaly presenting at the age of 35 years. Following transsphenoidal surgery, his IGF-1 normalized and clinical symptoms improved. He was found to have a novel AIP mutation destroying the stop codon c.991T>C; p.*331R. Unexpectedly, his father and paternal aunt were negative for this mutation while his mother and older sister were unaffected carriers, suggesting that his aunt represents a phenocopy.

  10. AHR over-expression in papillary thyroid carcinoma: clinical and molecular assessments in a series of Italian acromegalic patients with a long-term follow-up.

    Directory of Open Access Journals (Sweden)

    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.

  11. Comparing two acromegalic patients with respect to central corneal thickness, intraocular pressure, and tear insulin-like growth factor levels before and after treatment

    Directory of Open Access Journals (Sweden)

    Kan Emrah

    2015-01-01

    Full Text Available The aim of the study was to compare the central corneal thickness (CCT, intraocular pressure (IOP, and tear insulin-like growth factor-1 (IGF-1 levels of 2 patients with acromegaly before and after the surgical treatment of the disease. CCTs, IOP levels, and tear IGF-1 values showed a decrease after the treatment in 2 patients. As we found higher CCT, IOP, and tear IGF-1 levels in the active phase of the disease in two acromegaly patients, detailed information about the activity of the disease may be important before the examination of these patients.

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

    International Nuclear Information System (INIS)

    Gembicki, M.; Kosowicz, J.

    1978-01-01

    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)

  13. Low plasma lecithin : cholesterol acyltransferase and lipid transfer protein activities in growth hormone deficient and acromegalic men: role in altered high density lipoproteins

    NARCIS (Netherlands)

    Beentjes, JAM; van Tol, A; Sluiter, WJ; Dullaart, RPF

    2000-01-01

    Growth hormone (GH) deficiency and acromegaly may be associated with increased cardiovascular risk. Little is known about alterations in high density lipoproteins (HDL) in these conditions. Lecithin:cholesterol acyl transferase (LCAT) has the ability to esterify free cholesterol (FC) in HDL.

  14. Anaesthesia for transsphenoidal surgery in a patient with extreme gigantism.

    Science.gov (United States)

    Chan, V W; Tindal, S

    1988-03-01

    The management of anaesthesia for transsphenoidal removal of a pituitary adenoma in a true pituitary giant with acromegaly is described. Problems which may be anticipated in such a patient and an approach to their management are discussed, with particular emphasis upon the need for thorough preoperative assessment of the upper airway and the provision of adequate pulmonary ventilation during anaesthesia.

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

    NARCIS (Netherlands)

    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

  16. Fat deposition in the cavernous sinus in Cushing disease

    International Nuclear Information System (INIS)

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

    1984-01-01

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

  17. pituitary tumours, epidemiology, pathogenesis and management ...

    African Journals Online (AJOL)

    The management of pituitary tumours has advanced considerably over the last decade. A variety of novel dopamine agonists has revolutionised the management of prolactinomas, while the availability of effective somatostatin analogues has raised the possibility of primary medical treatment of acromegaly. Furthermore, the ...

  18. Author Details

    African Journals Online (AJOL)

    Non Piluitary Surgery in a patient with Acromegaly complicated by Dilated Cardiomyopathy: Anaesthetic challenges. Abstract · Vol 16, No 1 (2006) - Articles Resuscitation Guideline 2000: What is the level of Awareness and Knowledge in Practicing Anaesthestists? Abstract · Vol 16, No 3 (2006) - Articles Medical Students\\' ...

  19. Fat deposition in the cavernous sinus in Cushing disease

    Energy Technology Data Exchange (ETDEWEB)

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

    1984-10-01

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

  20. Author Details

    African Journals Online (AJOL)

    Faerber, EN. Vol 47, No 4 (1973) - Articles Acromegaly Presenting with Hemiplegia Abstract PDF. ISSN: 0256-95749. AJOL African Journals Online. HOW TO USE AJOL... for Researchers · for Librarians · for Authors · FAQ's · More about AJOL · AJOL's Partners · Terms and Conditions of Use · Contact AJOL · News. OTHER ...

  1. Effect of somatostatin on repair of ionizing radiation-induced DNA damage in pituitary adenoma cells GH3

    Czech Academy of Sciences Publication Activity Database

    Řezáčová, M.; Čáp, J.; Vokurková, D.; Lukášová, Emilie; Vávrová, J.; Cerman, J.; Mašín, V.; Mazánková, N.

    2008-01-01

    Roč. 57, č. 2 (2008), s. 225-235 ISSN 0862-8408 Institutional research plan: CEZ:AV0Z50040507; CEZ:AV0Z50040702 Keywords : acromegaly * somatostatin * ionizing radiation Subject RIV: BO - Biophysics Impact factor: 1.653, year: 2008

  2. West African Journal of Medicine - Vol 29, No 2 (2010)

    African Journals Online (AJOL)

    Pregnancy Associated with Recurrent Acromegaly: A Case Report · EMAIL FREE FULL TEXT EMAIL FREE FULL TEXT · DOWNLOAD FULL TEXT DOWNLOAD FULL TEXT. AE Edo, A Eregie. http://dx.doi.org/10.4314/wajm.v29i2.68207 ...

  3. Algorithmic complexity of growth hormone release in humans

    Energy Technology Data Exchange (ETDEWEB)

    Prank, K.; Wagner, M.; Brabant, G. [Medical School Hannover (Germany)

    1996-12-31

    Most hormones are secreted in an pulsatile rather than in a constant manner. This temporal pattern of pulsatile hormone release plays an important role in the regulation of cellular function and structure. In healthy humans growth hormone (GH) secretion is characterized by distinct pulses whereas patients bearing a GH producing tumor accompanied with excessive secretion (acromegaly) exhibit a highly irregular pattern of GH release. It has been hypothesized that this highly disorderly pattern of GH release in acromegaly arises from random events in the GH-producing tumor under decreased normal control of GH secretion. Using a context-free grammar complexity measure (algorithmic complexity) in conjunction with random surrogate data sets we demonstrate that the temporal pattern of GH release in acromegaly is not significantly different from a variety of stochastic processes. In contrast, normal subjects clearly exhibit deterministic structure in their temporal patterns of GH secretion. Our results support the hypothesis that GH release in acromegaly is due to random events in the GH-producing tumorous cells which might become independent from hypothalamic regulation. 17 refs., 1 fig., 2 tabs.

  4. Endocrine Hypertension | Elamin | Sudan Journal of Medical Sciences

    African Journals Online (AJOL)

    acromegaly, thyrotoxicosis, hypothyroidism, and hyperparathyroidism. Endocrine hypertension is noted in both adults and children but it is more relevant for children where it comes second to renal hypertension, the common cause of hypertension in that age group. Sudan Journal of Medical Sciences Vol. 3 (3) 2008: pp.

  5. Browse Title Index

    African Journals Online (AJOL)

    ... hemorrhage mimicking a pneumopathy: a rare complication of dual antiplatelet therapy for ST elevation myocardial infarction, Abstract PDF. Sara Oualim, Charafeddine Ait Elharda, Dounia Benzeroual, Mustapha El Hattaoui. Vol 12, No 1 (2012), Pulmonary epidermoid carcinoma in a patient with acromegaly: a rare entity ...

  6. L'acromégalie du sujet âgé: quelles particularités? | Anoun | Pan ...

    African Journals Online (AJOL)

    Surgery, if possible, is the treatment of choice for acromegaly in the elderly. Somatostatin analogues have shown to be effective in these patients. Prognosis is inversely correlated with patient's age, duration of disease and last GH level under treatment. Beside evolution of disease, age is a major determinant of mortality.

  7. Nigerian Quarterly Journal of Hospital Medicine - Vol 15, No 2 (2005)

    African Journals Online (AJOL)

    Non Piluitary Surgery in a patient with Acromegaly complicated by Dilated Cardiomyopathy: Anaesthetic challenges. EMAIL FULL TEXT EMAIL FULL TEXT DOWNLOAD FULL TEXT DOWNLOAD FULL TEXT. N A Merah, C I Okeke, A C Obikudu, 103-105. http://dx.doi.org/10.4314/nqjhm.v15i2.12766 ...

  8. Pathophysiology of the GH/IGF-1 axis : long-term consequences on joints and bone

    NARCIS (Netherlands)

    Claessen, Kim Maria Johanna Aldegonda

    2014-01-01

    In this thesis, a number of observations are described in acromegaly patients with cured or biochemically well-controlled disease during long-term follow-up. These observations focus on the long-term consequences of the disease on joints and bone. In addition, we investigated the role of the Growth

  9. Lessons learned from 15 years of KIMS and 5 years of ACROSTUDY

    DEFF Research Database (Denmark)

    Luger, Anton; Feldt-Rasmussen, Ulla; Abs, Roger

    2011-01-01

    -deficient adults for 15 years. Approximately 5 years ago, the ACROSTUDY database was established to monitor the long-term safety and effectiveness of pegvisomant in patients with acromegaly. CONCLUSIONS: By collecting data on the treatment of relatively rare conditions in routine clinical practice...

  10. 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

    DEFF Research Database (Denmark)

    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...

  11. Different effects of continuous and intermittent patterns of growth hormone administration on lipoprotein levels in growth hormone-deficient patients

    DEFF Research Database (Denmark)

    Laursen, Torben; Lemming, Lone; Jørgensen, Jens Otto Lunde

    1998-01-01

    with acromegaly. Studies in rats have demonstrated differential effects of constant and intermittent GH patterns on levels of certain lipoproteins. The aim of the present studies was to describe the impact of intermittent and continuous patterns of GH delivery to GHD patients on serum levels of Lp(a) and other...

  12. Clinical studies of IGFBP-2 by radioimmunoassay

    DEFF Research Database (Denmark)

    Blum, W F; Horn, N; Kratzsch, J

    1993-01-01

    and experimental situations. There was little circadian fluctuations of serum level which showed a marked age-dependence with high levels at birth and senescence and low levels during puberty. Decreased IGFBP-2 levels were present in untreated insulin-dependent diabetes mellitus (IDDM), in acromegaly and during...

  13. The Insulin-Sensitive Side of SHIP2

    Directory of Open Access Journals (Sweden)

    Stephane Schurmans

    2001-01-01

    Full Text Available A substantial and increasing proportion of death and disability in the EU (and elsewhere is attributable to diseases associated with insulin resistance (i.e., decreased insulin sensitivity. Beside type II diabetes, other diseases like obesity, hypertension, atherosclerosis, hyperlipidaemia, polycystic ovarian syndrome, and acromegaly are indeed associated with insulin resistance [1].

  14. Antiprogestins in Small Animal Reproduction - More than Induction of Abortion in Dogs

    DEFF Research Database (Denmark)

    Goericke-Pesch, Sandra Kathrin

    2017-01-01

    described for gestagen-dependent insulin resistant diabetes mellitus, acromegaly in bitches, size reduction of benign canine vaginal tumours and reduction of proliferation of progesterone-receptor expressing mammary carcinomas. This review aims to give an update on possibilities and indicates limitations...

  15. Pituitary diseases : long-term psychological consequences

    NARCIS (Netherlands)

    Tiemensma, Jitske

    2012-01-01

    Nowadays, pituitary adenomas can be appropriately treated, but patients continue to report impaired quality of life (QoL) despite long-term remission or cure. In patients with Cushing’s disease, Cushing’s syndrome or acromegaly, doctors should be aware of subtle cognitive impairments and the

  16. Growth hormone deficiency and hyperthermia during exercise

    DEFF Research Database (Denmark)

    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. WAJM 29(2) LATEST.pmd

    African Journals Online (AJOL)

    user1

    Pregnancy Associated with Recurrent Acromegaly: A Case Report. Associée à la grossesse avec récurrentes acromégalie: Un ... disorder. Pregnancy is an unusual event in acromegalic females because fertility is often reduced. .... Computed tomography. (CT) scan of brain showed no significant enlargement of sella turcica ...

  18. Pituitary magnetic resonance imaging is not required in the postoperative follow-up of acromegalic patients with long-term biochemical cure after transsphenoidal surgery

    NARCIS (Netherlands)

    Zirkzee, E. J. M.; Corssmit, E. P. M.; Biermasz, N. R.; Brouwer, P. A.; Wiggers-de Bruine, F. T.; Kroft, L. J. M.; van Buchem, M. A.; Roelfsema, F.; Pereira, A. M.; Smit, J. W. A.; Romijn, J. A.

    2004-01-01

    After successful transsphenoidal surgery for acromegaly, life-long follow-up is required, because 10-15% of patients develop recurrence of disease. We assessed whether it is safe to perform postoperative follow-up with only biochemical evaluation in acromegalic patients initially cured by

  19. Hypertension in Adults: Part 1. Prevalence, types, causes and effects

    African Journals Online (AJOL)

    Siegal_D

    Hyper / Hypothyroidism. ▫ Acromegaly. ▫ Hyperparathyroidism. ▫ Exogenous hormones, e.g. contraceptive pills, glucocorticoids. 2. Renal causes: ▫ Glomerulonephritis. ▫ Diabetic nephropathy. ▫ Polycystic kidney disease. ▫ Renal artery stenosis. 3. Other causes: ▫ Coarctation of the aorta. ▫ Pregnancy associated hypertension.

  20. Preoperative preparation of patients with pituitary gland disorders.

    Science.gov (United States)

    Malenković, Vesna; Gvozdenović, Ljiljana; Milaković, Branko; Sabljak, Vera; Ladjević, Nebojsa; Zivaljević, Vladan

    2011-01-01

    This paper presents the most common disorders of pituitary function: acromegaly, hypopituitarism, diabetes insipidus and syndrome similar to diabetes insipidus, in terms of their importance in preoperative preparation of patients. Pituitary function manages almost the entire endocrine system using the negative feedback mechanism that is impaired by these diseases. The cause of acromegaly is a pituitary adenoma, which produces growth hormone in adults. Primary therapy of acromegaly is surgical, with or without associated radiotherapy. If a patient with acromegaly as comorbidity prepares for non-elective neurosurgical operation, then it requires consultation with brain surgeons for possible delays of that operation and primary surgical treatment of pituitary gland. If operative treatment of pituitary gland is carried out, the preoperative preparation (for other surgical interventions) should consider the need for perioperative glucocorticoid supplementation. Panhypopituitarism consequences are different in children and adults and the first step in diagnosis is to assess the function of target organs. Change of electrolytes and water occurs in the case of pituitary lesions in the form of central or nephrogenic diabetes insipidus as a syndrome of inappropriate secretion of antidiuretic hormone (SIADH). Preoperative preparation of patients with pituitary dysfunction should be multidisciplinary, whether it is a neurosurgical or some other surgical intervention. The aim is to evaluate the result of insufficient production of pituitary hormones (hypopituitarism), excessive production of adenohypophysis hormones (acromegaly, Cushing's disease and hyperprolactinemia) and the influence of pituitary tumours in surrounding structures (compression syndrome) and to determine the level of perioperative risk. Pharmacological suppressive therapy of the hyperfunctional pituitary disorders can have significant interactions with drugs used in the perioperative period.

  1. Growth Hormone Research Society perspective on biomarkers of GH action in children and adults.

    Science.gov (United States)

    Johannsson, Gudmundur; Bidlingmaier, Martin; Biller, Beverly M K; Boguszewski, Margaret; Casanueva, Felipe F; Chanson, Philippe; Clayton, Peter E; Choong, Catherine S; Clemmons, David; Dattani, Mehul; Frystyk, Jan; Ho, Ken; Hoffman, Andrew R; Horikawa, Reiko; Juul, Anders; Kopchick, John J; Luo, Xiaoping; Neggers, Sebastian; Netchine, Irene; Olsson, Daniel S; Radovick, Sally; Rosenfeld, Ron; Ross, Richard J; Schilbach, Katharina; Solberg, Paulo; Strasburger, Christian; Trainer, Peter; Yuen, Kevin C J; Wickstrom, Kerstin; Jorgensen, Jens O L

    2018-03-01

    The Growth Hormone Research Society (GRS) convened a Workshop in 2017 to evaluate clinical endpoints, surrogate endpoints and biomarkers during GH treatment of children and adults and in patients with acromegaly. GRS invited 34 international experts including clinicians, basic scientists, a regulatory scientist and physicians from the pharmaceutical industry. Current literature was reviewed and expert opinion was utilized to establish the state of the art and identify current gaps and unmet needs. Following plenary presentations, breakout groups discussed questions framed by the planning committee. The attendees re-convened after each breakout session to share the group reports. A writing team compiled the breakout session reports into a document that was subsequently discussed and revised by participants. This was edited further and circulated for final review after the meeting. Participants from pharmaceutical companies were not part of the writing process. The clinical endpoint in paediatric GH treatment is adult height with height velocity as a surrogate endpoint. Increased life expectancy is the ideal but unfeasible clinical endpoint of GH treatment in adult GH-deficient patients (GHDA) and in patients with acromegaly. The pragmatic clinical endpoints in GHDA include normalization of body composition and quality of life, whereas symptom relief and reversal of comorbidities are used in acromegaly. Serum IGF-I is widely used as a biomarker, even though it correlates weakly with clinical endpoints in GH treatment, whereas in acromegaly, normalization of IGF-I may be related to improvement in mortality. There is an unmet need for novel biomarkers that capture the pleiotropic actions of GH in relation to GH treatment and in patients with acromegaly. © 2018 Growth Hormone Research Society.

  2. Pasireotide therapy in a rare and unusual case of plurihormonal pituitary macroadenoma.

    Science.gov (United States)

    Rajendran, Rajesh; Naik, Sarita; Sandeman, Derek D; Nasruddin, Azraai B

    2013-01-01

    We report the use of pasireotide in a rare and unusual case of pituitary macroadenoma co-secreting GH, prolactin and ACTH. A 62-year-old Caucasian man presented with impotence. Clinically, he appeared acromegalic and subsequent investigations confirmed GH excess and hyperprolactinaemia. Magnetic resonance imaging (MRI) of pituitary revealed a large pituitary macroadenoma. He underwent trans-sphenoidal surgery and histology confirmed an adenoma with immunohistochemistry positive for ACTH, GH and prolactin. Acromegaly was not cured following surgery and inadequately controlled despite subsequent octreotide therapy. He underwent further debulking pituitary surgery, following which IGF1 levels improved but still high. This time adenoma cells showed immunohistochemistry positivity for ACTH only, following which subsequent investigations confirmed intermittent hypercortisolaemia compatible with pituitary Cushing's disease. We recommended radiotherapy, but in view of the pluripotential nature of the tumour, we proceeded with a trial of s.c. pasireotide therapy on the basis that it may control both his acromegaly and Cushing's disease. After 3 months of pasireotide therapy, his mean GH and IGF1 levels improved significantly, with improvement in his symptoms but intermittent hypercortisolaemia persists. His glycaemic control deteriorated requiring addition of new anti-diabetic medication. MRI imaging showed loss of contrast uptake within the tumour following pasireotide therapy but no change in size. We conclude that our patient has had a partial response to pasireotide therapy. Long-term follow-up studies are needed to establish its safety and efficacy in patients with acromegaly and/or Cushing's disease. Plurihormonal pituitary adenomas are rare and unusual.Patients with pituitary adenomas co-secreting ACTH and GH are more likely to present with acromegaly because GH excess can mask hypercortisolaemia.Pasireotide holds potential where conventional somatostatin analogues

  3. Partial purification of Leucine aminopeptidase (LAP) in Acromegalic Sample of Iraqi Patients

    Science.gov (United States)

    Uloom Mohammad, Taghreed

    2018-05-01

    Acromagaly is a syndrome caused by increased growth hormone secretion from the frontal lobe of the pituitary gland. A Leucine aminopeptidase (EC 34111) activity has been assayed in (30) patients sera samples(15 female and 15 males) with acromegaly age range between (3050) years and (30) sera of healthy as control group (16 femal and 14 male) age range between (3050) years. The goal of the research was partial purified of enzyme from sera patients with acromegaly by dialysis gel filtration by using sephdex G50 and ion exchange chromatography by using DEAE cellulose A50. The results showed a single peak by using gel filtration and the activity was reached to 152 U/L. Two isoenzymes were obtained by using ion exchange chromatography and the purity degree of isoenzymse (I II) were (125) and (128) fold respectively. The current study found that the enzyme showed no significant difference between the healthy and the patients.

  4. Into the wardrobe of Narnia: beyond HIV infection a world of cardiovascular risk.

    Science.gov (United States)

    Colotto, Marco; Renzi, Alessandra; Durante, Cosimo

    2012-07-17

    A 38-years-old HIV-hepatitis C virus (HCV) coinfected woman presented to us for dyslipidemia. Clinical, familial and laboratory data were consistent with the diagnosis of familial heterozygous hypercholesterolaemia. History, clinical examination and laboratory findings also supported suspected acromegaly. A pituitary MRI showed a sellar macroadenoma that was removed by transphenoidal surgery without complications. MRI carried out 6 months after surgery showed almost complete removal of the adenoma. This case report describes an uncommon association of diseases, very different for aetiology--genetic for FH, infectious for HIV and HCV, caused by excessive hormone secretion for acromegaly--clinical features and therapy, but all sharing a significant impact on cardiovascular risk as a common feature.

  5. Las manifestaciones orales de acromegalia

    International Nuclear Information System (INIS)

    Chen Ku, Chih Hao; Alpizar Monge, Dafne

    2005-01-01

    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) [es

  6. Thyrotropin secreting pituitary adenoma accompanying a silent somatotropinoma.

    Science.gov (United States)

    Berker, Dilek; Isik, Serhat; Aydin, Yusuf; Tutuncu, Yasemin; Akdemir, Gokhan; Ozcan, Hatice Nursun; Guler, Serdar

    2011-01-01

    Thyroid stimulating hormone (TSH) secreting pituitary adenomas are rare tumors manifested as hyperthyroidism with goiter in the presence of elevated TSH. We present a case with pituitary adenoma secreting both TSH and growth hormone (GH) with the prominent clinical findings of hyperthyroidism but without clinical findings of acromegaly. Pituitary magnetic resonance imaging revealed a macroadenoma. Transsphenoidal surgery was performed twice. The immunohistochemical staining showed that tumor cells were strongly reactive to GH and relatively mildly reactive to TSH. Control pituitary imaging revealed a residual macroadenoma, and long acting octreotide treatment was administered. After two years of the treatment, tumor size remained the same while thyroid function tests and insulin-like growth factor 1 (IGF-I) values returned to normal ranges. In conclusion, we always recommend hormonal examinations for all patients who have pituitary adenoma without signs and symptoms of acromegaly.

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

    International Nuclear Information System (INIS)

    Gasser, R.W.; Finkenstedt, G.; Skrabal, F.

    1981-01-01

    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)

  8. Giant growth-hormone secreting pituitary tumour with etracranial extension

    Energy Technology Data Exchange (ETDEWEB)

    Ip Taipang; Chan Fuluk; Kung Annie Waichee; Lam Karen Siuling [Univ. of Hong Kong, Queen Mary Hospital (Hong Kong). Depts. of Medicine and Diagnostic Radiology

    1996-02-01

    A 19 year old female patient with typical features of acromegaly was found to have an extensive pituitary tumour with suprasellar, lateral and inferior extensions. Magnetic resonance imaging (MRI) also showed a portion of the tumour extending from the right cavernous sinus through the foramen ovale to become extracranial. Serum growth hormone (GH) was 52.6 mU/L basally and remained elevated after oral glucose, confirming the diagnosis of acromegaly. Treatment with the long-acting somatostatin analogue, octreotide, for 6 months led to a 30% reduction in tumour volume of the intracranial portion but no effect on the extracranial and sphenoidal extensions. She was subsequently treated with trans-sphenoidal surgery followed by external irradiation. The possibility of perineural spread of the tumour was considered. 9 refs., 1 tab., 1 fig.

  9. Giant growth-hormone secreting pituitary tumour with etracranial extension

    International Nuclear Information System (INIS)

    Ip Taipang; Chan Fuluk; Kung Annie Waichee; Lam Karen Siuling

    1996-01-01

    A 19 year old female patient with typical features of acromegaly was found to have an extensive pituitary tumour with suprasellar, lateral and inferior extensions. Magnetic resonance imaging (MRI) also showed a portion of the tumour extending from the right cavernous sinus through the foramen ovale to become extracranial. Serum growth hormone (GH) was 52.6 mU/L basally and remained elevated after oral glucose, confirming the diagnosis of acromegaly. Treatment with the long-acting somatostatin analogue, octreotide, for 6 months led to a 30% reduction in tumour volume of the intracranial portion but no effect on the extracranial and sphenoidal extensions. She was subsequently treated with trans-sphenoidal surgery followed by external irradiation. The possibility of perineural spread of the tumour was considered. 9 refs., 1 tab., 1 fig

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

    Energy Technology Data Exchange (ETDEWEB)

    Gasser, R.W.; Finkenstedt, G.; Skrabal, F. (Innsbruck Univ. (Austria). Medizinische Klinik)

    1981-01-01

    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.

  11. Clinical experience in the screening and management of a large kindred with familial isolated pituitary adenoma due to an aryl hydrocarbon receptor interacting protein (AIP) mutation.

    Science.gov (United States)

    Williams, Fred; Hunter, Steven; Bradley, Lisa; Chahal, Harvinder S; Storr, Helen L; Akker, Scott A; Kumar, Ajith V; Orme, Stephen M; Evanson, Jane; Abid, Noina; Morrison, Patrick J; Korbonits, Márta; Atkinson, A Brew

    2014-04-01

    Germline AIP mutations usually cause young-onset acromegaly with low penetrance in a subset of familial isolated pituitary adenoma families. We describe our experience with a large family with R304* AIP mutation and discuss some of the diagnostic dilemmas and management issues. The aim of the study was to identify and screen mutation carriers in the family. Forty-three family members participated in the study. The study was performed in university hospitals. We conducted genetic and endocrine screening of family members. We identified 18 carriers of the R304* mutation, three family members with an AIP-variant A299V, and two family members who harbored both changes. One of the two index cases presented with gigantism and pituitary apoplexy, the other presented with young-onset acromegaly, and both had surgery and radiotherapy. After genetic and clinical screening of the family, two R304* carriers were diagnosed with acromegaly. They underwent transsphenoidal surgery after a short period of somatostatin analog treatment. One of these two patients is in remission; the other achieved successful pregnancy despite suboptimal control of acromegaly. One of the A299V carrier family members was previously diagnosed with a microprolactinoma; we consider this case to be a phenocopy. Height of the unaffected R304* carrier family members is not different compared to noncarrier relatives. Families with AIP mutations present particular problems such as the occurrence of large invasive tumors, poor response to medical treatment, difficulties with fertility and management of pregnancy, and the finding of AIP sequence variants of unknown significance. Because disease mostly develops at a younger age and penetrance is low, the timing and duration of the follow-up of carriers without overt disease requires further study. The psychological and financial impact of prolonged clinical screening must be considered. Excellent relationships between the family, endocrinologists, and

  12. Scalloping at the lumbosacral canal

    Energy Technology Data Exchange (ETDEWEB)

    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.

  13. Individual Differences in Diabetes Risk: Role of Sleep Disturbances

    Science.gov (United States)

    2009-08-01

    In acromegaly, hypothyroidism and Cushing syndrome, the prevalence of OSA is 19%–23%, 50%–100%, and 18%–32%, respec- tively.43 The pathophysiology... hypothyroidism ”, “polycystic ovary syndrome”, and “PCOS”. We also searched the reference lists of identified articles for further papers. Articles were...Eur. Respir. J. 29, 720–727 (2007). 63. Trenell, M. I. et al. Influence of constant, positive airway pressure therapy on lipid storage, muscle

  14. ONR Tokyo Scientific Bulletin. Volume 4, Number 4, October-December 1979,

    Science.gov (United States)

    1979-12-01

    myotonic dystrophy, myxeodema, obesity , Parkinson’s disease, epilepsy, alcoholism, schizophrenia, rheumatoid arthritis, tetanus, acromegaly, depression...12 12 Iraq 2,000 8 7 Korea 2,000 84 26 Malaysia 3,000 5 5 Phillipines 5,000 18 10 Singapore 1,000 2 2 Sri Lanka 7,000 7 7 Thailand 2,000 18 10...previously in the Bulletin. MALAYSIA Chemistry research in Malaysian universities is mainly in the areas of applications such as natural products

  15. Scalloping at the lumbosacral canal

    International Nuclear Information System (INIS)

    Reinhardt, R.

    1987-01-01

    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. (orig.) [de

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

    Directory of Open Access Journals (Sweden)

    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.

  17. In-frame seven amino-acid duplication in AIP arose over the last 3000 years, disrupts protein interaction & stability and is associated with gigantism.

    OpenAIRE

    Salvatori, R.; Radian, S.; Diekmann, Y.; Iacovazzo, D.; David, A.; Grabovska, P.; Grassi, G.; Bussell, A-M; Stals, K.; Weber, A.; Quinton, R.; Crowne, E.; Corazzini, V.; Metherell, L. A.; Kearney, T.

    2017-01-01

    OBJECTIVE: Mutations in the aryl hydrocarbon receptor-interacting protein (AIP) gene are associated with pituitary adenoma, acromegaly and gigantism. Identical alleles in unrelated pedigrees could be inherited from a common ancestor or result from recurrent mutation events. DESIGN & METHODS: Observational, inferential and experimental study, including: AIP mutation testing; reconstruction of 14 AIP-region (8.3 Mbp) haplotypes; coalescent-based approximate Bayesian estimation of the time to mo...

  18. In-frame seven amino-acid duplication in AIP arose over the last 3000 years, disrupts protein interaction and stability and is associated with gigantism

    OpenAIRE

    Salvatori, Roberto; Radian, Serban; Diekmann, Yoan; Iacovazzo, Donato; David, Alessia; Gabrovska, Plamena; Grassi, Giorgia; Bussell, Anna-Marie; Stals, Karen; Weber, Astrid; Quinton, Richard; Crowne, Elizabeth C; Corazzini, Valentina; Metherell, Lou; Kearney, Tara

    2017-01-01

    Objective Mutations in the aryl hydrocarbon receptor-interacting protein (AIP) gene are associated with pituitary adenoma, acromegaly and gigantism. Identical alleles in unrelated pedigrees could be inherited from a common ancestor or result from recurrent mutation events. Design and methods Observational, inferential and experimental study, including: AIP mutation testing; reconstruction of 14 AIP-region (8.3?Mbp) haplotypes; coalescent-based approximate Bayesian estimation of the time to mo...

  19. Pituitary-hormone secretion by thyrotropinomas

    OpenAIRE

    Roelfsema, Ferdinand; Kok, Simon; Kok, Petra; Pereira, Alberto M.; Biermasz, Nienke R.; Smit, Jan W.; Frolich, Marijke; Keenan, Daniel M.; Veldhuis, Johannes D.; Romijn, Johannes A.

    2008-01-01

    Hormone secretion by somatotropinomas, corticotropinomas and prolactinomas exhibits increased pulse frequency, basal and pulsatile secretion, accompanied by greater disorderliness. Increased concentrations of growth hormone (GH) or prolactin (PRL) are observed in about 30% of thyrotropinomas leading to acromegaly or disturbed sexual functions beyond thyrotropin (TSH)-induced hyperthyroidism. Regulation of non-TSH pituitary hormones in this context is not well understood. We there therefore ev...

  20. Silent pituitary macroadenoma co-secreting growth hormone and thyroid stimulating hormone.

    Science.gov (United States)

    Sen, Orhan; Ertorer, M Eda; Aydin, M Volkan; Erdogan, Bulent; Altinors, Nur; Zorludemir, Suzan; Guvener, Nilgun

    2005-04-01

    Silent pituitary adenomas are a group of tumors showing heterogenous morphological features with no hormonal function observed clinically. To date no explanation has been provided as to why these tumors remain "silent". We report a case of a silent macroadenoma with both growth hormone (GH) and thyroid stimulating hormone (TSH) staining and secretion but with no clinical manifestations, in particular, the absence of features of acromegaly or hyperthyroidism. The relevant literature is reviewed.

  1. Concerning the etiology of bony bridges along the sides of the terminal phalanx of the great toe

    Energy Technology Data Exchange (ETDEWEB)

    Fischer, E.

    1987-06-01

    Besides in acromegaly bony bridges at the terminal phalanx of the great toe occur in one third of cases with peripheral signs of diffuse idiopathic skeletal hyperostosis, systemic diseases as rheumatoid arthritis or psoriatic arthritis with chronic inflammation of the interphalangeal joint of the great toe respectively extraarticular osseous changes in the terminal phalanx of the great toe do not influence the development of such bridges.

  2. Normal and pathologically altered oculomotoric muscles in CT picture

    Energy Technology Data Exchange (ETDEWEB)

    Kvicala, V.; Balakova, H. (Karlova Univ., Prague (Czechoslovakia). Fakulta Vseobecneho Lekarstvi)

    1984-03-01

    Computerized tomography reliably visualizes oculomotoric muscles, particularly in coronary projection. 21 patients were examined where computerized tomography of the orbit showed disorders of oculomotoric muscles. Thyreoprivic ophthalmopathy (8 patients) was manifest by non-symmetric irregular thickening of muscles, whose density was unhomogeneously higher. In acromegaly (3 patients) the thickening of the muscles was less, affecting all muscles to a similar degree. Inflammatory and tumorous processes always affected only one oculomotoric muscle.

  3. Surgery induced hypopituitarism in acromegalic patients: a systematic review and meta-analysis of the results.

    Science.gov (United States)

    Carvalho, Pedro; Lau, Eva; Carvalho, Davide

    2015-12-01

    Hypopituitarism is a possible complication of the surgical treatment of acromegaly. However, there is a wide variability in the incidence rates of surgery-induced hypopituitarism. The purpose of this study was the systematic collection and synthesis of information on the incidence rates of hypopituitarism, panhypopituitarism, specific axis deficiencies and diabetes insipidus after surgery for acromegaly treatment. We systematically reviewed all the papers that have reported pituitary deficits after surgery for acromegaly published up until December 2014, in the PubMed database. We identified 92 studies enrolling 6988 patients. A meta-analysis was performed to evaluate the incidence rates. We also performed several subgroup analyses to evaluate the impact of both surgical technique, and treatment prior to surgery, on the results. The weighted incidence rates were 12.79 % for hypopituitarism (95 % CI 9.88-16.00 %), 2.50 % for panhypopituitarism (95 % CI 1.24-4.15 %), 6.50 % for ACTH deficiency (95 % CI 4.07-9.44 %), 4.39 % for TSH deficiency (95 % CI 2.99-6.04 %), 6.70 % for FSH/LH deficiency (95 % CI 3.89-10.17 %), 14.95 % for GH deficiency (95 % CI 7.25-24.64 %), 10.05 % for transient (95 % CI 7.18-13.33 %) and 2.42 % for permanent diabetes insipidus (95 % CI 1.70-3.27 %). Our study provides new data on the incidence rates of hypopituitarism, specific pituitary axis deficiencies and diabetes insipidus after surgical treatment of acromegaly. Somatotroph function appears to be more prone to deficit than the other axes. However, there is a high heterogeneity between studies and several factors may influence the incidence of hypopituitarism.

  4. Impact of Growth Hormone on Cystatin C

    Directory of Open Access Journals (Sweden)

    Lisa Sze

    2013-11-01

    Full Text Available Background: Cystatin C (CysC is an alternative marker to creatinine for estimation of the glomerular filtration rate (GFR. Hormones such as thyroid hormones and glucocorticoids are known to have an impact on CysC. In this study, we examined the effect of growth hormone (GH on CysC in patients with acromegaly undergoing transsphenoidal surgery. Methods: Creatinine, CysC, GH and insulin-like growth factor-1 (IGF-1 were determined in 24 patients with acromegaly before and following transsphenoidal surgery. Estimated GFR was calculated using the Chronic Kidney Disease Epidemiology Collaboration formula. Results: In all patients, surgical debulking resulted in decreased clinical disease activity and declining GH/IGF-1 levels. Postoperatively, biochemical cure was documented in 20 out of 24 patients. Creatinine levels (mean ± SEM increased from 72 ± 3 to 80 ± 3 µmol/l (p = 0.0004 and concurrently, estimated GFR decreased from 99 ± 3 to 91 ± 3 ml/min (p = 0.0008. In contrast to creatinine, CysC levels decreased from 0.72 ± 0.02 to 0.68 ± 0.02 mg/l (p = 0.0008. Conclusions: Our study provides strong evidence for discordant effects of GH on creatinine and CysC in patients with acromegaly undergoing transsphenoidal surgery, thus identifying another hormone that influences CysC independent of renal function.

  5. Higher Income and Integration into the Workforce Are the Main Factors Associated with Quality of Life in Acromegalic Patients in Northeastern Brazil.

    Science.gov (United States)

    GuimarãesSá, Adriana Maria; Ferreira, Pedro Antônio Muniz; Souza, Marinilde Teles; Nascimento, Gilvan Cortês; da Silva Pereira Damianse, Sabrina; de Carvalho Rocha, Viviane Chaves; Dos Santos Faria, Manuel; de Souza Paiva Ferreira, Adalgisa

    2018-01-01

    To identify the factors associated with quality of life in patients with acromegaly with follow-up at the referral service in neuroendocrinology of the state of Maranhão, northeast Brazil. The Acromegaly Quality of Life Questionnaire (Acro-QoL) was used. Factors independently associated with quality of life were identified using multivariate linear regression, with p values integrated into the job market and quality of life scores in the overall domain ( β = 0.288, p = 0.003), psychological domain ( β = 0.291, p = 0.032), and personal relationship domain ( β = 0.314, p = 0.019). We also observed a positive association with income and the quality of life scores in all domains as follows: overall domain ( β = 0.037, p = 0.003), physical domain ( β = 0.988, p = 0.001), psychological domain ( β = 0.342, p = 0.008), physical appearance domain ( β = 0.270, p = 0.049), and personal relationship domain ( β = 0.315, p = 0.012). For patients with acromegaly living in one of the least developed regions of Brazil, integration into the job market and a higher income were associated with a better quality of life.

  6. Quantitative evaluation of indium-111 (In-111) octreotide pituitary activity: Comparison in patient with and without pituitary tumors

    Energy Technology Data Exchange (ETDEWEB)

    Gupta, P.; Waxman, A.; Nguyen, K. [Cedars-Sinai Medical Center, Los Angeles, CA (United States)] [and others

    1995-05-01

    Indium 111 Octreotide is known to detect pituitary tumors. Variable low level pituitary activity has been reported in pts. with no demonstrable pituitary tumors. To our knowledge, there have been no studies which quantitatively categorize pituitary activity with respect to distinguishing normal subject from pts. with pituitary tumors. 13 pts. with proven, treated acromegaly were included, as well as 15 pts. with no history of pituitary disorder. Both groups underwent SPECT In-111 scintigraphy 24 hours post-injection Average count per pixel ratios were obtained for the pituitary/calvarium (P/C) and pituitary/brain (P/B) regions. 10 pts. with acromegaly underwent growth hormone (GH) measurements 2 hours post-glucose load. Statistical correlation between growth hormone levels using P/C and P/B ratios were obtained. P/C ratios, as well as P/B ratios demonstrated high correlation with serum GH levels correlation coefficient(r)= .717 for P/C p<0.05, and correlation coefficient(r) = 0.828 for P/B ratios p<0.005. P/C ratios and P/B ratios for controls correlated closely with the upper level of normal predicted by P/C or P/B ratios as a function of serum growth hormone found in patients with acromegaly. Somatostatin receptor SPECT scintigraphy of the pituitary and appropriate quantitation can predict patients with growth hormone secreting tumors.

  7. A novel germline mutation in the aryl hydrocarbon receptor-interacting protein (AIP) gene in an Italian family with gigantism.

    Science.gov (United States)

    Urbani, C; Russo, D; Raggi, F; Lombardi, M; Sardella, C; Scattina, I; Lupi, I; Manetti, L; Tomisti, L; Marcocci, C; Martino, E; Bogazzi, F

    2014-10-01

    Acromegaly usually occurs as a sporadic disease, but it may be a part of familial pituitary tumor syndromes in rare cases. Germline mutations in the aryl hydrocarbon receptor-interacting protein (AIP) gene have been associated with a predisposition to familial isolated pituitary adenoma. The aim of the present study was to evaluate the AIP gene in a patient with gigantism and in her relatives. Direct sequencing of AIP gene was performed in fourteen members of the family, spanning among three generations. The index case was an 18-year-old woman with gigantism due to an invasive GH-secreting pituitary adenoma and a concomitant tall-cell variant of papillary thyroid carcinoma. A novel germline mutation in the AIP gene (c.685C>T, p.Q229X) was identified in the proband and in two members of her family, who did not present clinical features of acromegaly or other pituitary disorders. Eleven subjects had no mutation in the AIP gene. Two members of the family with clinical features of acromegaly refused either the genetic or the biochemical evaluation. The Q229X mutation was predicted to generate a truncated AIP protein, lacking the last two tetratricopeptide repeat domains and the final C-terminal α-7 helix. We identified a new AIP germline mutation predicted to produce a truncated AIP protein, lacking its biological properties due to the disruption of the C-terminus binding sites for both the chaperones and the client proteins of AIP.

  8. Unusual AIP mutation and phenocopy in the family of a young patient with acromegalic gigantism.

    Science.gov (United States)

    Imran, Syed Ali; Aldahmani, Khaled A; Penney, Lynette; Croul, Sidney E; Clarke, David B; Collier, David M; Iacovazzo, Donato; Korbonits, Márta

    2018-01-01

    Early-onset acromegaly causing gigantism is often associated with aryl-hydrocarbon-interacting receptor protein ( AIP ) mutation, especially if there is a positive family history. A15y male presented with tiredness and visual problems. He was 201 cm tall with a span of 217 cm. He had typical facial features of acromegaly, elevated IGF-1, secondary hypogonadism and a large macroadenoma. His paternal aunt had a history of acromegaly presenting at the age of 35 years. Following transsphenoidal surgery, his IGF-1 normalized and clinical symptoms improved. He was found to have a novel AIP mutation destroying the stop codon c.991T>C; p.*331R. Unexpectedly, his father and paternal aunt were negative for this mutation while his mother and older sister were unaffected carriers, suggesting that his aunt represents a phenocopy. Typical presentation for a patient with AIP mutation with excess growth and eunuchoid proportions.Unusual, previously not described AIP variant with loss of the stop codon.Phenocopy may occur in families with a disease-causing germline mutation.

  9. Endocrinologist's first experience in the treatment of pituitary adenomas using Leksell's gamma knife

    International Nuclear Information System (INIS)

    Marek, J.; Malik, J.; Fendrych, P.

    1996-01-01

    The group treated included 13 patients with pituitary adenomas. Hormonally active acromegaly was found in 9 patients, prolactinoma in 1 patient, and afunctional adenoma in 3 patients. Twelve patients had previously undergone surgery, 3 of them twice. Magnetic resonance was used for imaging the pituitary prior to the surgery and one year later. The following hormonal factors were examined: the growth hormone during the day and during the thyroliberin test, the insulin-like growth factor I, the prolactin level, the thyrotropin level during the thyrotropin-releasing hormone test, the thyroxine and triiodothyronine levels, the plasma concentration of adrenocorticotropin, the cortisol level, the plasma testosterone level, and the 17-beta estradiol level. Complete recovery within 18 months after the Leksell surgery was only achieved in 1 female patient with acromegaly. In other 5 patients with acromegaly and 1 patient with prolactinoma, the hormonal levels decreased partly without demonstrable changes in the size of the adenoma. Hypopituarism developed in 1 only female patient within 18 months after the irradiation. No other complications were observed. Stereotactic irradiation with Leksell's gamma knife proved to be a useful and well tolerated approach in the treatment of pituitary adenomas. 11 refs

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

    Directory of Open Access Journals (Sweden)

    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

  11. Studying Cat (Felis catus Diabetes: Beware of the Acromegalic Imposter.

    Directory of Open Access Journals (Sweden)

    Stijn J M Niessen

    Full Text Available Naturally occurring diabetes mellitus (DM is common in domestic cats (Felis catus. It has been proposed as a model for human Type 2 DM given many shared features. Small case studies demonstrate feline DM also occurs as a result of insulin resistance due to a somatotrophinoma. The current study estimates the prevalence of hypersomatotropism or acromegaly in the largest cohort of diabetic cats to date, evaluates clinical presentation and ease of recognition. Diabetic cats were screened for hypersomatotropism using serum total insulin-like growth factor-1 (IGF-1; radioimmunoassay, followed by further evaluation of a subset of cases with suggestive IGF-1 (>1000 ng/ml through pituitary imaging and/ or histopathology. Clinicians indicated pre-test suspicion for hypersomatotropism. In total 1221 diabetic cats were screened; 319 (26.1% demonstrated a serum IGF-1>1000 ng/ml (95% confidence interval: 23.6-28.6%. Of these cats a subset of 63 (20% underwent pituitary imaging and 56/63 (89% had a pituitary tumour on computed tomography; an additional three on magnetic resonance imaging and one on necropsy. These data suggest a positive predictive value of serum IGF-1 for hypersomatotropism of 95% (95% confidence interval: 90-100%, thus suggesting the overall hypersomatotropism prevalence among UK diabetic cats to be 24.8% (95% confidence interval: 21.2-28.6%. Only 24% of clinicians indicated a strong pre-test suspicion; most hypersomatotropism cats did not display typical phenotypical acromegaly signs. The current data suggest hypersomatotropism screening should be considered when studying diabetic cats and opportunities exist for comparative acromegaly research, especially in light of the many detected communalities with the human disease.

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

    Directory of Open Access Journals (Sweden)

    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. IGF-1 levels may increase paradoxically with dopamine agonist treatment for prolactinomas.

    Science.gov (United States)

    Akirov, Amit; Greenman, Yona; Glaser, Benjamin; S'chigol, Irena; Mansiterski, Yossi; Eizenberg, Yoav; Shraga-Slutzky, Ilana; Shimon, Ilan

    2018-05-04

    Hyperprolactinemia is common in acromegaly and in these patients, insulin-like growth factor (IGF)-1 level may decrease with dopamine agonist. We report a series of patients with prolactinoma and a paradoxical increase of IGF-1 levels during cabergoline treatment. Clinical characteristics and response to treatment of patients with prolactinomas, in whom normal or slightly elevated baseline IGF-1 levels increased with cabergoline. The cohort consisted of ten prolactinoma patients (nine males, mean age 48 ± 14 years). Mean adenoma size was 23.8 ± 16.2 mm, with cavernous sinus invasion in eight. In five patients baseline IGF-1 levels were normal and in four levels were 1.2-1.5-fold the upper limit of the normal (ULN). One patient had IGF-1 measured shortly after initiating cabergoline and it was 1.4 × ULN. During cabergoline treatment (dose range 0.5-2 mg/week) PRL normalization was achieved in all and tumor shrinkage occurred in seven patients. The mean IGF-1 increase on cabergoline was 1.7 ± 0.4 × ULN. Cabergoline dose reduction or interruption was attempted in five patients and resulted in decreased IGF-1 levels in all, including normalization in two patients. Three patients were eventually diagnosed with acromegaly, one was referred for pituitary surgery followed by complete remission, another patient was switched to somatostatin analogue, and the third was treated by combination of somatostatin analogues with pegvisomant, with reduction of IGF-1 in all these patients. IGF-1 levels may increase to clinically significant levels during cabergoline treatment for PRL-adenoma. We suggest IGF-1 monitoring in all patients treated with dopamine agonists and not only in those presenting symptoms of acromegaly.

  14. In-frame seven amino-acid duplication in AIP arose over the last 3000 years, disrupts protein interaction and stability and is associated with gigantism.

    Science.gov (United States)

    Salvatori, Roberto; Radian, Serban; Diekmann, Yoan; Iacovazzo, Donato; David, Alessia; Gabrovska, Plamena; Grassi, Giorgia; Bussell, Anna-Marie; Stals, Karen; Weber, Astrid; Quinton, Richard; Crowne, Elizabeth C; Corazzini, Valentina; Metherell, Lou; Kearney, Tara; Du Plessis, Daniel; Sinha, Ajay Kumar; Baborie, Atik; Lecoq, Anne-Lise; Chanson, Philippe; Ansorge, Olaf; Ellard, Sian; Trainer, Peter J; Balding, David; Thomas, Mark G; Korbonits, Márta

    2017-09-01

    Mutations in the aryl hydrocarbon receptor-interacting protein ( AIP ) gene are associated with pituitary adenoma, acromegaly and gigantism. Identical alleles in unrelated pedigrees could be inherited from a common ancestor or result from recurrent mutation events. Observational, inferential and experimental study, including: AIP mutation testing; reconstruction of 14 AIP -region (8.3 Mbp) haplotypes; coalescent-based approximate Bayesian estimation of the time to most recent common ancestor (tMRCA) of the derived allele; forward population simulations to estimate current number of allele carriers; proposal of mutation mechanism; protein structure predictions; co-immunoprecipitation and cycloheximide chase experiments. Nine European-origin, unrelated c.805_825dup-positive pedigrees (four familial, five sporadic from the UK, USA and France) included 16 affected (nine gigantism/four acromegaly/two non-functioning pituitary adenoma patients and one prospectively diagnosed acromegaly patient) and nine unaffected carriers. All pedigrees shared a 2.79 Mbp haploblock around AIP with additional haploblocks privately shared between subsets of the pedigrees, indicating the existence of an evolutionarily recent common ancestor, the 'English founder', with an estimated median tMRCA of 47 generations (corresponding to 1175 years) with a confidence interval (9-113 generations, equivalent to 225-2825 years). The mutation occurred in a small tandem repeat region predisposed to slipped strand mispairing. The resulting seven amino-acid duplication disrupts interaction with HSP90 and leads to a marked reduction in protein stability. The c.805_825dup allele, originating from a common ancestor, associates with a severe clinical phenotype and a high frequency of gigantism. The mutation is likely to be the result of slipped strand mispairing and affects protein-protein interactions and AIP protein stability. © 2017 The authors.

  15. Growth Hormone and Endocrinopathies

    Energy Technology Data Exchange (ETDEWEB)

    Kim, K W; Choe, K O; Park, C Y; Lee, H; Son, H Y; Huh, K B; Ryu, K J [Yonsei University College of Medicine, Seoul (Korea, Republic of)

    1979-03-15

    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. Growth Hormone and Endocrinopathies

    Energy Technology Data Exchange (ETDEWEB)

    Kim, K. W.; Choe, K. O.; Park, C. Y.; Lee, H.; Son, H. Y.; Huh, K. B.; Ryu, K. J. [Yonsei University College of Medicine, Seoul (Korea, Republic of)

    1979-03-15

    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.

  17. Growth Hormone and Endocrinopathies

    International Nuclear Information System (INIS)

    Kim, K. W.; Choe, K. O.; Park, C. Y.; Lee, H.; Son, H. Y.; Huh, K. B.; Ryu, K. J.

    1979-01-01

    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.

  18. Present Concepts in Internal Medicine. Volume 13, Number 1. Endocrinology Research Symposium,

    Science.gov (United States)

    1980-01-01

    osteoporosis 5t 41 M 0.99 0.91 Idiopathic osteoporosis of 10 yrs. duration 6 22 F 0.87 1.97 Diabetes melitus , idopathicstress fractures 7 25 F 1.58...If neceesry and Identify by block number) Cushing’s disease; Acromegaly; Secretory pituitary adenomata; Vitamin D; Somatomedin; Diabetes Mellitus...computer in the management and study of diabetes mellitus; studies of the trophi properties of gastrin on the gut; and gastrin in the human small bowel. It

  19. Coexistence of GH-Producing Pituitary Macroadenoma and Meningioma in a Patient with Multiple Endocrine Neoplasia Type 1 with Hyperglycemia and Ketosis as First Clinical Sign

    Directory of Open Access Journals (Sweden)

    A. Herrero-Ruiz

    2017-01-01

    Full Text Available We present the clinical case of a patient who was admitted with an onset of diabetes mellitus (DM with associated ketosis and whose clinical, hormonal, and radiological evolution revealed the presence of primary hyperparathyroidism, pancreatic neuroendocrine tumor, and GH-producing pituitary macroadenoma in the context of multiple endocrine neoplasia type 1 (MEN1. DM is relatively common in cases of acromegaly, but it is not generally associated with ketosis. Simultaneously, the patient presented a meningioma, which is associated with pituitary macroadenoma only in extremely rare cases.

  20. Transsphenoidal Surgery for Pituitary Tumors and Other Sellar Masses.

    Science.gov (United States)

    Owen, Tina J; Martin, Linda G; Chen, Annie V

    2018-01-01

    Transsphenoidal surgery is an option for dogs and cats with functional and nonfunctional pituitary masses or other sellar and parasellar masses. An adrenocorticotropic hormone-secreting tumor causing Cushing disease is the most common clinically relevant pituitary tumor in dogs, and the most common pituitary tumor seen in cats is a growth hormone-secreting tumor causing acromegaly. Transsphenoidal surgery can lead to rapid resolution of clinical signs and provide a cure for these patients. Because of the risks associated with this surgery, it should only be attempted by a cohesive pituitary surgery group with a sophisticated medical and surgical team. Copyright © 2017 Elsevier Inc. All rights reserved.