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    Berardinelli-Seip Congenital Generalised Lipodystrophy.
    J Coll Physicians Surg Pak 2018 May;28(5):406-408
    Department of Pediatric Gastroenterology and Hepatology, The Children's Hospital and The Institute of Child Health, Lahore.
    Berardinelli-Seip congenital lipodystrophy (BSCL) syndrome is a rare genetic disorder caused by dysregulation of glycemic and lipid metabolism. We report five BSCL cases with typical clinical pictures and complications. These, to the best of our knowledge, represent the first case series from Pakistan. Read More

    Pituitary Diseases and Bone.
    Endocr Rev 2018 Apr 19. Epub 2018 Apr 19.
    Chair of Endocrinology, Università Vita-Salute San Raffaele, Milan, Italy.
    Neuroendocrinology of bone is a new area of research based on the evidence that pituitary hormones may directly modulate bone remodeling and metabolism. Skeletal fragility associated with high risk of fractures is a common complication of several pituitary diseases such as hypopituitarism, Cushing disease, acromegaly and hyperprolactinemia. As in other forms of secondary osteoporosis, pituitary diseases generally affect bone quality more than bone quantity and fractures may occur even in the presence of normal or low-normal bone mineral density as measured by dual-energy X-ray absorptiometry, making difficult the prediction of fractures in these clinical settings. Read More

    Higher Income and Integration into the Workforce Are the Main Factors Associated with Quality of Life in Acromegalic Patients in Northeastern Brazil.
    Int J Endocrinol 2018 27;2018:6135080. Epub 2018 Feb 27.
    Graduate Program in Public Health, Department of Public Health, UFMA, São Luís, MA, Brazil.
    Objective: 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.

    Methods: The Acromegaly Quality of Life Questionnaire (Acro-QoL) was used. Factors independently associated with quality of life were identified using multivariate linear regression, with values < 0. Read More

    Cystic tumors of the pituitary infundibulum: seminal autopsy specimens (1899 to 1904) that allowed clinical-pathological craniopharyngioma characterization.
    Pituitary 2018 Apr 21. Epub 2018 Apr 21.
    Department of Pathology and Clinical Bacteriology, Jakob Erdheim Institute, Vienna, Austria.
    A heterogeneous group of epithelial cystic tumors developed at the infundibulum and the third ventricle disconcerted pathologists at the dawn of the twentieth century. Very little was known at that time about the physiological role played by the pituitary gland, and there was almost complete ignorance regarding the function of the hypothalamus. Acromegaly, or enlargement of acral body parts, described in 1886 by Pierre Marie, was the only disease linked to primary hypertrophies of the pituitary gland, known as "pituitary strumas". Read More

    Endocrine Remission After Pituitary Stereotactic Radiosurgery: Differences in Rates of Response for Matched Cohorts of Cushing Disease and Acromegaly Patients.
    Int J Radiat Oncol Biol Phys 2018 Feb 20. Epub 2018 Feb 20.
    Department of Neurological Surgery, University of Virginia, Charlottesville, Virginia; Department of Radiation Oncology, University of Virginia, Charlottesville, Virginia.
    Purpose: To compare and describe the time to endocrine remission and new hypopituitarism among patients with growth hormone (GH) and adrenocorticotropic hormone (ACTH)-secreting pituitary adenomas after radiosurgery, controlling for several known prognostic factors.

    Methods And Materials: An institutional review board-approved, institutional retrospective analysis of patients with GH- and ACTH-secreting pituitary adenomas was performed, with matching for patient sex, age at radiosurgery, interval between the last resection and radiosurgery, use of previous radiation therapy, whole sella treatment, suprasellar extension, cavernous sinus invasion, and margin dose. Endocrine remission was defined as a normal serum insulin-like growth factor-1 (GH secreting) or a normal 24-hour urine-free cortisol (ACTH secreting) level without suppressive medications. Read More

    An orphan G-protein-coupled receptor causes human gigantism and/or acromegaly: Molecular biology and clinical correlations.
    Best Pract Res Clin Endocrinol Metab 2018 Apr 17;32(2):125-140. Epub 2018 Mar 17.
    Section on Endocrinology and Genetics (SEGEN), Eunice Kennedy Shriver National Institute of Child Health and Human Development (NICHD), National Institutes of Health (NIH), Bethesda, MD, 20892-1862, USA. Electronic address:
    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. Read More

    Association between radiological parameters and clinical and molecular characteristics in human somatotropinomas.
    Sci Rep 2018 Apr 18;8(1):6173. Epub 2018 Apr 18.
    Maimonides Institute of Biomedical Research of Cordoba, Córdoba, 14004, Spain.
    Acromegaly is a rare but severe disease, originated in 95% of cases by a growth hormone-secreting adenoma (somatotropinoma) in the pituitary. Magnetic resonance imaging (MRI) is a non-invasive technique used for the diagnosis and prognosis of pituitary tumours. The aim of this study was to determine whether the use of T2-weighted signal intensity at MRI could help to improve the characterisation of somatotropinomas, by analysing its relationship with clinical/molecular features. Read More

    Non-adenomatous pituitary tumours mimicking functioning pituitary adenomas.
    Br J Neurosurg 2018 Apr 18:1-5. Epub 2018 Apr 18.
    b Key Laboratory of Pituitary Adenoma in Guangdong Province, Department of Neurosurgery , First Affiliated Hospital, SunYat-sen University , Guangzhou , China.
    Objective: Pituicytomas and granular cell tumours (GCTs) of the neurohypophysis are considered non-adenomatous neoplasms in the sellar region. The association between hormone hypersecretion and the tumours is seldom discussed and unclear. Therefore, we attempt to investigate this association based on our experience and a review of the literature. Read More

    Iran Pituitary Tumor Registry: Description of the Program and Initial Results.
    Arch Iran Med 2017 Dec 31;20(12):746-751. Epub 2017 Dec 31.
    Endocrine Research Center, Institute of Endocrinology and Metabolism, Iran University of Medical Sciences (IUMS), Tehran, Iran.
    Background: This study was designed to present initial results on clinical presentation, therapeutic modalities, and outcome information of patients with pituitary tumors registered in Iran Pituitary Tumor Registry (IPTR).

    Methods: We collected data from a web-based electronic medical records of patients with various pituitary tumors referred to four tertiary care centers in the country. Retrospective analysis was performed on demographic, clinical, and therapeutic information of 298 patients including 51 clinically nonfunctioning adenoma (CNFA), 85 acromegaly, 135 prolactinoma, and 27 Cushing's disease (CD). Read More

    [Association between diabetic ketoacidosis and acromegaly].
    Medicina (B Aires) 2018 ;78(2):131-133
    Departamento de Medicina, VI Cátedra de Medicina, Hospital de Clínicas José de San Martin, Universidad de Buenos Aires, Argentina.
    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. Read More

    Acromegaly: A rare disease?
    Medicina (B Aires) 2018 ;78(2):83-85
    Fundación de Endocrinología (FUNDAENDO), Buenos Aires, Argentina. E-mail:
    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. Read More

    Criteria for diagnosis and postoperative control of acromegaly, and screening and management of its comorbidities: Expert consensus.
    Endocrinol Diabetes Nutr 2018 Apr 10. Epub 2018 Apr 10.
    Hospital Universitario Dexeus, Barcelona, España.
    Acromegaly is a rare disease with many comorbidities that impair quality of life and limit survival. There are discrepancies in various clinical guidelines regarding diagnosis and postoperative control criteria, as well as screening and optimal management of comorbidities. This expert consensus was aimed at establishing specific recommendations for the Spanish healthcare system. Read More

    Cognitive-behavioral therapy improves the quality of life of patients with acromegaly.
    Pituitary 2018 Apr 11. Epub 2018 Apr 11.
    SRTVS 701, Edifício Centro Empresarial Brasília, Bloco "C", sala 204, Asa Sul, Brasília, DF, CEP: 70.340-907, Brazil.
    Background: The delayed diagnosis, altered body image, and clinical complications associated with acromegaly impair quality of life.

    Purpose: To assess the efficacy of the cognitive-behavioral therapy (CBT) technique "Think Healthy" to increase the quality of life of patients with acromegaly.

    Methods: This non-randomized clinical trial examined ten patients with acromegaly (nine women and one man; mean age, 55. Read More

    Association of clinical and laboratory parameters with ambulatory arterial stiffness index in acromegaly patients.
    Pak J Med Sci 2018 Jan-Feb;34(1):37-42
    Alpaslan Kemal Tuzcu, Department of Endocrinology, Medical Faculty, Dicle University, Diyarbakır, Turkey.
    Objective: In this study, we determined the relationship between the ambulatory arterial stiffness index (AASI) and clinical and laboratory parameters in patients with acromegaly.

    Methods: Sixty-five patients with acromegaly, who visited to Dicle University Medical Faculty Department of Endocrinology (33 females and 32 males), were included in this study. The study control group consisted of 65 subjects. Read More

    Impact of Glucose Metabolism Disorders on IGF-1 Levels in Patients with Acromegaly.
    Horm Metab Res 2018 Apr 11. Epub 2018 Apr 11.
    Istanbul University, Istanbul Faculty of Medicine, Division of Endocrinology and Metabolism, Istanbul, Turkey.
    In this study, we aimed to evaluate the presence of glucose metabolism abnormalities and their impact on IGF-1 levels in patients with acromegaly. Ninety-three patients with acromegaly (n=93; 52 males/41 females) were included in this study. Patients were separated into three groups such as; normal glucose tolerance (n=23, 25%), prediabetes (n=38, 41%), and diabetes mellitus (n=32, 34%). Read More

    Predictors of surgical outcome and early criteria of remission in acromegaly.
    Endocrine 2018 Apr 6. Epub 2018 Apr 6.
    Neuroendocrinology Research Center / Endocrinology Division - Medical School and Hospital Universitário Clementino Fraga Filho - Universidade Federal do Rio de Janeiro, Rio de Janeiro, Brazil.
    Background: Transsphenoidal surgery (TSS) is the cornerstone of acromegaly treatment, however there are no robust predictors of surgical outcome and remission can only be defined three months after surgery.

    Purpose: To analyze if biochemical, demographical, radiological, and immunohistochemical characteristics are predictors of surgical remission and investigate if immediate postoperative GH and IGF-I levels can help defining remission earlier.

    Methods: Consecutive acromegaly patients submitted to TSS between 2013-2016 were evaluated. Read More

    Craniofacial Manifestations of Systemic Disorders: CT and MR Imaging Findings and Imaging Approach.
    Radiographics 2018 Apr 6:170145. Epub 2018 Apr 6.
    From the Departments of Radiology (V.C.A.A., M.N.C., H.K., A.F., O.S.), Otolaryngology-Head and Neck Surgery (O.S.), and Radiation Oncology (O.S.), Boston University Medical Center, Boston University School of Medicine, 820 Harrison Ave, 3rd Floor, Boston, MA 02118.
    Many systemic diseases or conditions can affect the maxillofacial bones; however, they are often overlooked or incidentally found at routine brain or head and neck imaging performed for other reasons. Early identification of some conditions may significantly affect patient care and alter outcomes. Early recognition of nonneoplastic hematologic disorders, such as thalassemia and sickle cell disease, may help initiate earlier treatment and prevent serious complications. Read More

    USE OF ELECTRONIC HEALTH RECORDS TO CHARACTERIZE A RARE DISEASE IN THE USA: TREATMENT, COMORBIDITIES AND FOLLOW-UP TRENDS AMONG PATIENTS WITH A CONFIRMED DIAGNOSIS OF ACROMEGALY.
    Endocr Pract 2018 Apr 6. Epub 2018 Apr 6.
    Pfizer Inc, Rare Disease Medical Affairs.
    Purpose Understanding of acromegaly disease management is hampered in the US by lack of a national registry. We describe medical management in a population with confirmed acromegaly. Methods Inpatient and outpatient Electronic Health Records (EHR) were used to create a database of de-identified patients assigned the Acromegaly and Gigantism ICD-9 code and/or an appropriate pituitary procedure code at one of four regional hospital systems over a 6-11 year period. Read More

    A rare challenging case of co-existent craniopharyngioma, acromegaly and squamous cell lung cancer.
    Endocrinol Diabetes Metab Case Rep 2018 28;2018. Epub 2018 Mar 28.
    Institute of Metabolism and Systems Research, College of Medical and Dental Sciences, University of Birmingham, Birmingham, UK.
    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. Read More

    Persistence of Diabetes and Hypertension after Multimodal Treatment of Acromegaly.
    J Clin Endocrinol Metab 2018 Mar 30. Epub 2018 Mar 30.
    From the Endocrinology Service and the Experimental Endocrinology Unit, Hospital de Especialidades, Centro Médico Nacional Siglo XXI, Instituto Mexicano del Seguro Social, Mexico City.
    Context: Diabetes and hypertension are frequent comorbidities of acromegaly.

    Objective: To analyze the course of diabetes and hypertension at diagnosis and after multimodal therapy in a large cohort of patients with acromegaly.

    Design And Setting: Retrospective study, at a tertiary care center. Read More

    How are growth hormone and insulin-like growth factor-1 reported as markers for drug effectiveness in clinical acromegaly research? A comprehensive methodologic review.
    Pituitary 2018 Mar 31. Epub 2018 Mar 31.
    Division of Systems Biomedicine and Pharmacology, Leiden Academic Centre for Drug Research, Leiden University, Leiden, The Netherlands.
    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 biochemical control between studies. The aim of this review was to investigate the current methods that are being applied to measure and report growth hormone (GH) and insulin-like growth factor-1 (IGF-1) as markers for drug effectiveness in clinical acromegaly research. Read More

    Expression of the long non-coding RNA H19 and MALAT-1 in growth hormone-secreting pituitary adenomas and its relationship to tumor behavior.
    Int J Dev Neurosci 2018 Jun 28;67:46-50. Epub 2018 Mar 28.
    Department of Neurosurgery, The Affiliated Drum Tower Hospital of Nanjing University Medical School, 321 Zhongshan Road, Nanjing, 210008, Jiangsu, China. Electronic address:
    Aggressive growth hormone-secreting pituitary adenomas (GHPAs) represent an important clinical problem in patients with acromegaly. Surgical therapy, although often the mainstay of treatment for GHPAs, is less effective in aggressive GHPAs due to their invasive and destructive growth patterns, and their proclivity for infrasellar invasion. LncRNAs are important players in cancer development and emerging in various fundamental biological processes. Read More

    Biomarkers of GH action in children and adults.
    Growth Horm IGF Res 2018 Mar 20;40:1-8. Epub 2018 Mar 20.
    Department of Endocrinology and Internal Medicine, Aarhus University Hospital, Aarhus, Denmark.
    Growth hormone (GH) and IGF-I levels in serum are used as biomarkers in the diagnosis and management of GH-related disorders but have not been subject to structured validation. Auxological parameters in children and changes in body composition in adults, as well as metabolic parameters and patient related outcomes are used as clinical and surrogate endpoints. New treatment options, such as long acting GH and GH antagonists, require reevaluation of the currently used biochemical biomarkers. Read More

    Time spent with cats is never wasted: Lessons learned from feline acromegalic cardiomyopathy, a naturally occurring animal model of the human disease.
    PLoS One 2018 29;13(3):e0194342. Epub 2018 Mar 29.
    Clinical Science and Services, Royal Veterinary College, Hatfield, United Kingdom.
    Background: In humans, acromegaly due to a pituitary somatotrophic adenoma is a recognized cause of increased left ventricular (LV) mass. Acromegalic cardiomyopathy is incompletely understood, and represents a major cause of morbidity and mortality. We describe the clinical, echocardiographic and histopathologic features of naturally occurring feline acromegalic cardiomyopathy, an emerging disease among domestic cats. Read More

    Pasireotide - mechanism of action and clinical applications.
    Curr Drug Metab 2018 Mar 27. Epub 2018 Mar 27.
    Department of Endocrinology, Metabolism, and Internal Medicine, Faculty of Medicine II, Poznan University of Medical Sciences, Poznan. Poland.
    Pasireotide (SOM230) is a multi-receptor ligand somatostatin analogue (SSA) developed as the successor of the first-generation SSAs. Currently, pasireotide is recommended for the treatment of patients with Cushing's disease in whom surgery was unsuccessful, and patients with acromegaly who either remain uncontrolled after surgical therapy or in whom tumor resection is not possible. Phase II and III clinical trials have shown pasireotide efficacy in these diseases, with a similar rate of adverse events when compared with first-line SSA, although higher incidence of hyperglycemia has been observed. Read More

    GH, IGF-1, and Age Are Important Contributors to Thyroid Abnormalities in Patients with Acromegaly.
    Int J Endocrinol 2018 15;2018:6546832. Epub 2018 Jan 15.
    Department of Neurosurgery, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing 100730, China.
    Purpose: To determine the prevalence, risk factors, and possible mechanisms of structural and endocrinological changes to the thyroid in acromegaly.

    Methods: We studied 93 acromegalic patients from PUMCH between January 2013 and December 2013. The demographic and clinical information were recorded. Read More

    Cancer Incidence in Patients with Acromegaly: A cohort study and meta-analysis of the literature.
    J Clin Endocrinol Metab 2018 Mar 23. Epub 2018 Mar 23.
    Department of Endocrinology and Internal Medicine, Aarhus University Hospital, Aarhus, Denmark.
    Context: Acromegaly has been associated with increased risk of cancer morbidity and mortality, but research findings remain conflicting and population-based data are scarce. We therefore examined whether patients with acromegaly are at higher risk of cancer.

    Design: A nationwide cohort study (1978-2010) including 529 acromegaly cases was performed. Read More

    Somatostatin Analogs and Glucose Metabolism in Acromegaly: A Meta-analysis of Prospective Interventional Studies.
    J Clin Endocrinol Metab 2018 Mar 23. Epub 2018 Mar 23.
    Department of Experimental Medicine, Sapienza University of Rome, Italy.
    Introduction: Somatostatin analogs (SSAs) effectivelycontrol growth hormone secretion in first and second line treatmentof acromegaly. Their effect onglucose metabolism is still debated.

    Aim: to address the following questions: 1) Do SSAs affect fasting plasma glucose (FPG), fasting plasma insulin (FPI), glycosylated hemoglobin (HbA1c), glucose load (2h-OGTT), HOMA-I, HOMA-β, triglycerides (TGD), weight (W) or body mass index (BMI)? 2) Do lanreotide (LAN) and octreotide LAR (OCT) affect metabolism differently? 3)Does their effect depend on disease control?

    Methods: We performed a meta-analysis of prospective interventional trialstreating acromegaly with SSAs. Read More

    Endonasal endoscopic surgery in pituitary adenomas: Surgical results in a series of 86 consecutive patients.
    Neurocirugia (Astur) 2018 Mar 23. Epub 2018 Mar 23.
    Servicio de Otorrinolaringología, Hospital General Universitario de Alicante, Alicante, España.
    Introduction: The endoscopic endonasal approach has become the gold standard for the surgical treatment of pituitary adenomas.

    Objectives: The aim of this study is to present the results obtained in our hospital in purely endoscopic surgery of pituitary adenomas.

    Methods: From February 2011 to August 2016, we conducted a prospective study on a series of 86 patients with pituitary adenoma, all of whom underwent surgery with a purely endoscopic endonasal approach. Read More

    Outcome of pregnancies in a large cohort of women with acromegaly.
    Clin Endocrinol (Oxf) 2018 Mar 25. Epub 2018 Mar 25.
    Neuroendocrine Unit, Division of Endocrinology and Metabolism, Hospital das Clínicas, University of São Paulo Medical School, São Paulo, Brazil.
    Objective: To assess the outcome of pregnancies in a large cohort of women with acromegaly.

    Design And Methods: This is a retrospective analysis of 31 pregnancies in 20 patients with acromegaly.

    Results: Twenty-seven pregnancies resulted in healthy offspring, and 4 resulted in abortion. Read More

    Acromegalic cardiomyopathy: Epidemiology, diagnosis, and management.
    Clin Cardiol 2018 Mar 25;41(3):419-425. Epub 2018 Mar 25.
    Divison of Cardiovascular Medicine, University of California Davis Medical Center, Sacramento, California.
    Acromegalic cardiomyopathy is the leading cause of morbidity and all-cause mortality in patients with acromegaly. Though acromegaly is a rare condition, the associated derangements are vast and severe. Stemming from an increase in circulating growth hormone (GH) and insulin-like growth factor-1 levels (IGF-1), acromegalic cardiomyopathy results in pathological changes in myocyte growth and structure, cardiac contractility, and vascular function. Read More

    Three-dimensional speckle tracking echocardiography-derived left atrial deformation analysis in acromegaly (Results from the MAGYAR-Path Study).
    Echocardiography 2018 Mar 22. Epub 2018 Mar 22.
    2nd Department of Medicine and Cardiology Centre, Medical Faculty, Albert Szent-Györgyi Clinical Center, University of Szeged, Szeged, Hungary.
    Introduction: Acromegaly is a chronic and disfiguring hormonal disease caused by a benign growth hormone-secreting pituitary adenoma in most of the cases. The objective of this study was to investigate three-dimensional (3D) speckle tracking echocardiography (3DSTE)-derived left atrial (LA) volumetric and functional properties in patients with acromegaly. It was also examined whether the activity of acromegaly is associated with further alterations in these LA parameters. Read More

    Novel Somatostatin Receptor Ligands Therapies for Acromegaly.
    Front Endocrinol (Lausanne) 2018 7;9:78. Epub 2018 Mar 7.
    Department of Medicine, Division of Endocrinology, Metabolism and Diabetes, Pituitary Center Johns Hopkins University School of Medicine, Baltimore, MD, United States.
    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. Read More

    Value of early postoperative random growth hormone levels and nadir growth hormone levels after oral glucose tolerance testing in acromegaly.
    Growth Horm IGF Res 2018 Mar 8. Epub 2018 Mar 8.
    Department of Neurosurgery, University Medical Center Hamburg-Eppendorf, Martinistr. 52, 20246 Hamburg, Germany. Electronic address:
    Objective: There is no ideal marker to identify residual tumor tissue after surgery in patients with acromegaly. The purpose was to elucidate if early postoperative hormone testing gives reliable information regarding complete resection of a GH-producing pituitary adenoma.

    Design: Fourty-eight patients undergoing surgery for acromegaly from 04/2013-05/2014 were prospectively examined for random GH, IGF1, and GH levels after oral glucose tolerance testing (OGTT) in the early postoperative phase and on follow-up. Read More

    Does IGF-1 play a role in the etiopathogenesis of non-functioning adrenocortical adenoma?
    J Endocrinol Invest 2018 Mar 14. Epub 2018 Mar 14.
    Department of Endocrinology and Metabolism, Faculty of Medicine, Ondokuz Mayis University, Kurupelit, 55139, Samsun, Turkey.
    Purpose: The aim of this study was to investigate the possible association of insulin-like growth factor-1 (IGF-1) with the pathogenesis of non-functioning adrenocortical adenomas (NFAs).

    Methods: This study included 50 female patients (mean age 54 years) with NFAs, 55 patients (mean age 48 years; 20 male, 35 female) with acromegaly and 38 female control subjects (mean age 58 years). Body mass index (BMI) and waist circumference (WC) of the subjects were recorded and blood samples for IGF-1 were taken. Read More

    Pre- and Postoperative Body Composition and Metabolic Characteristics in Patients with Acromegaly: A Prospective Study.
    Int J Endocrinol 2018 8;2018:4125013. Epub 2018 Jan 8.
    Department of Neurosurgery, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, No. 1 Shuaifuyuan, Dongcheng District, Beijing 100730, China.
    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). Read More

    Cellular effects of AP102, a somatostatin analog with balanced affinities for the hSSTR2 and hSSTR5 receptors.
    Neuropeptides 2018 Apr 2;68:84-89. Epub 2018 Mar 2.
    Service of Clinical Pharmacology, Catecholamine and Peptides Laboratory, Centre Hospitalier Universitaire Vaudois (CHUV), 1001 Lausanne, Switzerland. Electronic address:
    Background: Somatostatin analogs (SSAs) are first-line medical therapy for the treatment of acromegaly and neuroendocrine tumors that express somatostatin receptors (SSTR). Somatostatin suppresses secretion of a large number of hormones through the stimulation of the five SSTR. However, unbalanced inhibition of secretion as observed with the highly potent SSAs pasireotide causes hyperglycaemia mainly by inhibiting insulin secretion. Read More

    Treatment escape reduces the effectiveness of cabergoline during long-term treatment of acromegaly in monotherapy or in association with first-generation somatostatin receptor ligands.
    Clin Endocrinol (Oxf) 2018 Mar 9. Epub 2018 Mar 9.
    Neuroendocrinology Research Center /Endocrinology Division, Medical School and Hospital Universitário Clementino Fraga Filho, Universidade Federal do Rio de Janeiro, Rio de Janeiro, Brazil.
    Background: Few studies evaluated the use of cabergoline (CAB) for acromegaly treatment in monotherapy or in combination with first-generation somatostatin receptor ligands (SRLs).

    Aim: To evaluate the efficacy, predictors of response and safety of CAB treatment in acromegaly both in monotherapy and in combination with SRLs.

    Methods: We retrospectively collected demographic, biochemical, tumour and treatment data. Read More

    Oro-dental pathologies in acromegaly.
    Endocrine 2018 May 8;60(2):323-328. Epub 2018 Mar 8.
    Department of Neurosurgery, Friedrich-Alexander University (FAU) of Erlangen-Nuremberg, Schwabachanlage 6, 91031, Erlangen, Germany.
    Purpose: Oro-dental pathologies (ODP) such as enlargement of the tongue, mandibular prognathism, and spaced teeth are characteristic features of acromegaly. Their frequency of occurrence during the course of the disease is largely unresolved. Purpose of this study was to assess ODP and oro-dental treatments in patients with acromegaly with regard to the length of the diagnostic process, tumor histology, and quality of life (QoL). Read More

    Ultrasound Elastography of the Median Nerve in Patients With Acromegaly: A Case-Control Study.
    J Ultrasound Med 2018 Mar 8. Epub 2018 Mar 8.
    Department of Endocrinology and Metabolism, Faculty of Medicine, Kirikkale University, Kirikkale, Turkey.
    Objectives: The aim of this study was to compare the cross-sectional area and ultrasound elastographic findings of the median nerve of patients with acromegaly and healthy participants.

    Methods: The acromegaly group comprised 15 patients with a previous diagnosis of acromegaly, and an age- and sex-matched control group included healthy participants. Strain elastographic and shear wave elastographic findings and the cross-sectional areas of the groups were compared. Read More

    Pachydermoperiostosis in a patient with chronic hepatitis B virus infection referred as acromegaly: a case report.
    J Med Case Rep 2018 Mar 8;12(1):59. Epub 2018 Mar 8.
    Department of Medicine and Therapeutics, School of Medicine and Dentistry, College of Health Sciences, University of Ghana, Legon, Accra, Ghana.
    Background: Primary hypertrophic osteoarthropathy also known as pachydermoperiostosis is a rare genetic disorder that has often been confused with acromegaly because of similar clinical features. Vascular endothelial growth factors which have been implicated in the clinical features of pachydermoperiostosis, have also been shown to be present in chronic hepatitis and implicated in the malignant transformation of hepatitis B infection to hepatocellular carcinoma. To the best of our knowledge there is one reported case of pachydermoperiostosis with chronic hepatitis B infection. Read More

    Multi-chaperone function modulation and association with cytoskeletal proteins are key features of the function of AIP in the pituitary gland.
    Oncotarget 2018 Feb 11;9(10):9177-9198. Epub 2018 Jan 11.
    Centre for Endocrinology, Barts and The London School of Medicine, Queen Mary University of London, London, EC1M 6BQ, UK.
    Despite the well-recognized role of loss-of-function mutations of the aryl hydrocarbon receptor interacting protein gene () predisposing to pituitary adenomas, the pituitary-specific function of this tumor suppressor remains an enigma. To determine the repertoire of interacting partners for the AIP protein in somatotroph cells, wild-type and variant AIP proteins were used for pull-down/quantitative mass spectrometry experiments against lysates of rat somatotropinoma-derived cells; relevant findings were validated by co-immunoprecipitation and co-localization. Global gene expression was studied in mutation positive and negative pituitary adenomas via RNA microarrays. Read More

    Radiotherapy in acromegaly: Long-term brain parenchymal and vascular magnetic resonance changes.
    J Neuroradiol 2018 Mar 2. Epub 2018 Mar 2.
    Neuroradiology, University of Salerno, via San Leonardo 1, 84131 Salerno, Italy.
    Background: Radiation therapy (RT) effectiveness on hormonal reduction is proven in acromegaly; however, collateral long-term effects are still undetermined. This transversal neuroimaging study on a large cohort of acromegalic patients aimed to investigate the rate of parenchymal and vascular changes after RT.

    Materials And Methods: Thirty-six acromegalic patients underwent RT (RT+) after unsuccessful surgery and were compared to RT- acromegalic patients matched for age, gender, adenoma features, clinical and surgical history. Read More

    Plurihormonal ACTH-GH Pituitary Adenoma: Case Report and Systematic Literature Review.
    World Neurosurg 2018 Mar 6. Epub 2018 Mar 6.
    Department of Medical and Surgical Specialties, Radiological Sciences, and Public Health, University of Brescia, Brescia, Italy. Electronic address:
    Background: Plurihormonal adenomas (PHAs) represent 10%-15% of all functioning pituitary adenomas. The most frequent hormonal associations are with prolactin and growth hormone (GH). Here we describe a rare case of functional adrenocorticotropic hormone (ACTH) and GH microadenoma and report our findings from a systematic literature review of PHA. Read More

    Decreasing mortality and changes in treatment patterns in patients with acromegaly from a nationwide study.
    Eur J Endocrinol 2018 May 26;178(5):459-469. Epub 2018 Feb 26.
    Department of EndocrinologyInstitute of Medicine, Sahlgrenska Academy, University of Gothenburg and Sahlgrenska University Hospital, Gothenburg, Sweden
    Context: New therapeutic strategies have developed for the management of acromegaly over recent decades. Whether this has improved mortality has not been fully elucidated.

    Objective: The primary aim was to investigate mortality in a nationwide unselected cohort of patients with acromegaly. Read More

    Growth Hormone Research Society perspective on biomarkers of GH action in children and adults.
    Endocr Connect 2018 Mar 26;7(3):R126-R134. Epub 2018 Feb 26.
    Aarhus University HospitalAarhus, Denmark
    Objective: 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.

    Participants: GRS invited 34 international experts including clinicians, basic scientists, a regulatory scientist and physicians from the pharmaceutical industry.

    Evidence: Current literature was reviewed and expert opinion was utilized to establish the state of the art and identify current gaps and unmet needs. Read More

    Postoperative Changes in Metabolic Parameters of Patients with Surgically Controlled Acromegaly: Assessment of New Stringent Cure Criteria.
    Neurol Med Chir (Tokyo) 2018 Apr 23;58(4):147-155. Epub 2018 Feb 23.
    Department of Neurosurgery, Graduate School of Medical and Dental Sciences, Kagoshima University.
    The criteria for surgical cure of acromegaly have become more stringent during the past decades and a change from Cortina to new consensus criteria has recently been proposed. However, the superiority of the new consensus over Cortina criteria with respect to postoperative metabolic parameters remains to be ascertained. We retrospectively assessed metabolic parameters, the body habitus, and other health-related parameters of 48 patients with surgically controlled acromegaly who met the Cortina criteria [normalized insulin-like growth factor-1 (IGF-1) level and nadir growth hormone (GH) level <1. Read More

    Correction to: Effects of pegvisomant and somatostatin receptor ligands on incidence of vertebral fractures in patients with acromegaly.
    Pituitary 2018 Feb 23. Epub 2018 Feb 23.
    Pituitary Unit, Catholic University of the Sacred Heart, Rome, Italy.
    The original version of the article contained an error in the results section of the Abstract. The vertebral fractures (VFs) odds ratio is incorrectly published as 61.0 and the correct value is 6. Read More

    Unusual mutation and phenocopy in the family of a young patient with acromegalic gigantism.
    Endocrinol Diabetes Metab Case Rep 2018 9;2018. Epub 2018 Feb 9.
    Centre for Endocrinology, Barts and the London School of Medicine, Queen Mary University of London, London, UK.
    Early-onset acromegaly causing gigantism is often associated with aryl-hydrocarbon-interacting receptor protein () 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. Read More

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