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Methodist DeBakey Cardiovascular Journal 2017In 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... (Review)
Review
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.
Topics: Acromegaly; Cardiovascular Diseases; Cardiovascular System; Human Growth Hormone; Humans; Insulin-Like Growth Factor I; Prognosis; Risk Factors
PubMed: 28740584
DOI: 10.14797/mdcj-13-2-64 -
The Journal of Clinical Endocrinology... Jun 2014McCune-Albright syndrome (MAS) includes the triad of poly/monostotic fibrous dysplasia, café-au-lait spots, and hyperfunctioning endocrinopathies. Acromegaly affects... (Review)
Review
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.
Topics: Acromegaly; Adolescent; Adult; Child; Child, Preschool; Female; Fibrous Dysplasia, Polyostotic; Humans; Male; Middle Aged; Young Adult
PubMed: 24517150
DOI: 10.1210/jc.2013-3826 -
Archives of Endocrinology and Metabolism 2019Acromegaly is a systemic disease associated with increased morbidity, presenting cardiovascular, metabolic, respiratory, neoplastic, endocrine, articular and bone... (Review)
Review
Acromegaly is a systemic disease associated with increased morbidity, presenting cardiovascular, metabolic, respiratory, neoplastic, endocrine, articular and bone complications. Most of these comorbidities can be prevented or delayed with adequate disease treatment and, more recent studies with the use of modern treatments of acromegaly, have shown a change in the severity and prevalence of these complications. In addition, acromegaly is associated with increased mortality, but recent studies (especially those published in the last decade) have shown a different scenario than older studies, with mortality no longer being increased in adequately controlled patients and a change in the main cause of death from cardiovascular disease to malignancy. In this review, we discuss this changing face of acromegaly summarizing current knowledge and evidence on morbimortality of the disease. Arch Endocrinol Metab. 2019;63(6):630-7.
Topics: Acromegaly; Cause of Death; Humans
PubMed: 31939488
DOI: 10.20945/2359-3997000000193 -
Reviews in Endocrine & Metabolic... Dec 2020Acromegaly is characterized by Growth Hormone (GH) and Insulin-like Growth Factor 1 (IGF-1) excess. Uncontrolled acromegaly is associated with a strongly increased risk... (Review)
Review
Acromegaly is characterized by Growth Hormone (GH) and Insulin-like Growth Factor 1 (IGF-1) excess. Uncontrolled acromegaly is associated with a strongly increased risk of cardiovascular disease (CVD), and numerous cardiovascular risk factors remain present after remission. GH and IGF-1 have numerous effects on the immune and cardiovascular system. Since endothelial damage and systemic inflammation are strongly linked to the development of CVD, and have been suggested to be present in both controlled as uncontrolled acromegaly, they may explain the presence of both micro- and macrovascular dysfunction in these patients. In addition, these changes seem to be only partially reversible after remission, as illustrated by the often reported presence of endothelial dysfunction and microvascular damage in controlled acromegaly. Previous studies suggest that insulin resistance, oxidative stress, and endothelial dysfunction are involved in the development of CVD in acromegaly. Not surprisingly, these processes are associated with systemic inflammation and respond to GH/IGF-1 normalizing treatment.
Topics: Acromegaly; Animals; Cardiovascular Diseases; Endothelium, Vascular; Human Growth Hormone; Humans; Inflammation; Insulin-Like Growth Factor I
PubMed: 32458292
DOI: 10.1007/s11154-020-09560-x -
Hormones (Athens, Greece) 2011Patients with acromegaly have characteristic clinical features caused by soft tissue overgrowth. The most common cause of acromegaly is a growth hormone-secreting...
Patients with acromegaly have characteristic clinical features caused by soft tissue overgrowth. The most common cause of acromegaly is a growth hormone-secreting adenoma of the anterior pituitary. Both somatic and metabolic features of acromegaly are due to excess growth hormone (GH) secretion and high serum concentrations of insulin-like growth factor-I (IGF-I). Here we present a case of "pseudoacromegaly" with an acromegaloid phenotype, insulin resistance, history of adenomatous colonic polyp, and suppressed IGF-I levels. Patients with this rare condition are likely to have a selective post-receptor defect of insulin signalling, leading to the impairment of metabolic but preservation of mitogenic signalling. Endocrinologists should consider this diagnosis when assessing patients with clinical features of acromegaly and insulin resistance, in the absence of elevated levels of GH and IGF-I.
Topics: Acromegaly; Female; Human Growth Hormone; Humans; Insulin; Insulin Resistance; Insulin-Like Growth Factor I; Middle Aged
PubMed: 21724541
DOI: 10.14310/horm.2002.1306 -
Frontiers in Endocrinology 2022Acromegaly is a rare condition typically caused by benign pituitary adenomas, resulting in excessive production of growth hormone. Clinical manifestations of acromegaly... (Review)
Review
Acromegaly is a rare condition typically caused by benign pituitary adenomas, resulting in excessive production of growth hormone. Clinical manifestations of acromegaly are diverse, varying from the overgrowth of body tissue to cardiovascular, metabolic, and osteoarticular disorders. Symptoms may emerge slowly, overlapping with other diseases and often involve many different healthcare specialists. In the last decade, efforts to provide an accurate and timely diagnosis of acromegaly have improved disease management and clinical experience. Despite this progress, marked differences in the diagnosis, treatment, and management of acromegaly exist from country-to-country. To address these inconsistencies in the region comprising Central and Eastern Europe, Israel, and Kazakhstan, a panel of acromegaly experts from 13 of these countries was convened. Acromegaly experts from each country provided available information on the approaches from their country, including regional treatment centers and multidisciplinary teams, treatment access, reimbursement and availability, and physician education, disease awareness, and patient advocacy. Across several areas of acromegaly management, divergent approaches were identified and discussed, including the provision of multidisciplinary care, approved and available treatments, and disease awareness programs. These were recognized as areas of potential improvement in the management of acromegaly, in addition to participation in national and regional acromegaly registries. Further experience exchange will facilitate the identification of specific strategies that can be adapted in each country, and widespread participation in acromegaly registries will enable their evaluation. It is anticipated that this approach will support the optimization of acromegaly patient care across this region.
Topics: Acromegaly; Europe, Eastern; Growth Hormone; Humans; Israel; Kazakhstan
PubMed: 35273565
DOI: 10.3389/fendo.2022.816426 -
Journal of Endocrinological... Oct 2022Acromegaly is a rare chronic disease characterized by systemic comorbidity and reduced quality of life. Although achieving biochemical control has always been the... (Review)
Review
PURPOSE
Acromegaly is a rare chronic disease characterized by systemic comorbidity and reduced quality of life. Although achieving biochemical control has always been the primary goal of acromegaly therapy, recent evidence has shown that the traditional assessment does not adequately capture the complexity of symptoms and patients' perception. These findings result in the need to improve a fast decision-making process of the clinician, who should not only take into account biochemical-instrumental criteria, but also patients' symptoms. With the aim of supporting the clinician in the diagnostic and therapeutic decision-making process several disease-specific tools have been developed. The aim of this review is to provide a description of the acromegaly-specific tools, presenting their main features, their application in daily practice, and their efficacy and utility.
METHODS
A systematic search of Medline/PubMed, ISI-Web of Knowledge, and Google Scholar databases was done.
RESULTS
Specific instruments and questionnaires have recently been developed to assist clinicians in the assessment of acromegaly. These are either Patient-Reported Outcome tools, such as Acromegaly Quality of Life Questionnaire (AcroQoL) and Pain Assessment Acromegaly Symptom Questionnaire (PASQ), or Clinician-Reported Outcome tools, such as ACROSCORE, SAGIT and Acromegaly Disease Activity Tool (ACRODAT). Such tools are extremely flexible and, therefore, have been widely adopted by endocrinologists and other professionals, so much so that they have also been included as recommendations in the 2018 international guidelines.
CONCLUSION
Questionnaires and tools are useful in the management of acromegaly patients. They help clinicians evaluate patients' symptoms and could assist in the evaluation of disease activity.
Topics: Acromegaly; Comorbidity; Databases, Factual; Humans; Quality of Life; Surveys and Questionnaires
PubMed: 35322391
DOI: 10.1007/s40618-022-01782-x -
Archives of Endocrinology and Metabolism 2019Acromegaly is an insidious disease, usually resulting from growth hormone hypersecretion by a pituitary adenoma. It is most often diagnosed during the 3rd to 4th decade... (Review)
Review
Acromegaly is an insidious disease, usually resulting from growth hormone hypersecretion by a pituitary adenoma. It is most often diagnosed during the 3rd to 4th decade of life. However, recent studies have shown an increase in the incidence and prevalence of acromegaly in the elderly, probably due to increasing life expectancy. As in the younger population with acromegaly, there is a delay in diagnosis, aggravated by the similarities of the aging process with some of the characteristics of the disease. As can be expected elderly patients with acromegaly have a higher prevalence of comorbidities than younger ones. The diagnostic criteria are the same as for younger patients. Surgical treatment of the pituitary adenoma is the primary therapy of choice unless contraindicated. Somatostatin receptor ligands are generally effective as both primary and postoperative treatment. The prognosis correlates inversely with the patient's age, disease duration and last GH level. Arch Endocrinol Metab. 2019;63(6):638-45.
Topics: Acromegaly; Aged; Aged, 80 and over; Female; Humans; Male; Prognosis
PubMed: 31939489
DOI: 10.20945/2359-3997000000194 -
The Journal of Clinical Endocrinology... Apr 2023Paltusotine is a once-daily, oral, nonpeptide small-molecule somatostatin receptor type 2 (SST2) agonist in clinical development for treatment of acromegaly. (Clinical Trial)
Clinical Trial
CONTEXT
Paltusotine is a once-daily, oral, nonpeptide small-molecule somatostatin receptor type 2 (SST2) agonist in clinical development for treatment of acromegaly.
OBJECTIVE
This work aimed to evaluate change in insulin-like growth factor I (IGF-I) levels in patients switched from octreotide long-acting release or lanreotide depot monotherapy to paltusotine.
METHODS
A phase 2, open-label, prospective, multicenter, multinational, nonrandomized, single-arm exploratory study was conducted in which dosage uptitrations were performed in a double-blinded manner. At 26 global sites, patients with acromegaly switched to paltusotine from injected somatostatin receptor ligand (SRL)-based therapy. Patients received 13-week treatment with once-daily oral paltusotine (10-40 mg/d). The primary end point was change from baseline to week 13 in IGF-I for patients who switched from long-acting octreotide or lanreotide depot monotherapy to paltusotine (group 1). All patients underwent a 4-week paltusotine washout at end of treatment period (wk 13-17). IGF-I, growth hormone (GH), patient-reported outcome, and safety data were collected.
RESULTS
Forty-seven patients enrolled. In group 1 (n = 25), IGF-I and GH showed no significant change between SRL baseline and end of paltusotine treatment at week 13 (median change in IGF-I = -0.03×upper limit of normal [ULN]; P = .6285; GH = -0.05 ng/mL; P = .6285). IGF-I and GH rose significantly in the 4 weeks after withdrawing paltusotine (median change in IGF-I = 0.55×ULN; P < .0001 [median increase 39%]; GH = 0.72 ng/mL; P < .0001 [109.1% increase]). No patients discontinued because of adverse events (AE); no treatment-related serious AEs were reported.
CONCLUSION
These results suggest once-daily oral paltusotine was effective in maintaining IGF-I values in patients with acromegaly who switched from injected SRLs. Paltusotine was well tolerated with a safety profile consistent with other SRLs.
Topics: Humans; Acromegaly; Octreotide; Insulin-Like Growth Factor I; Prospective Studies; Peptides, Cyclic; Human Growth Hormone; Treatment Outcome
PubMed: 36353760
DOI: 10.1210/clinem/dgac643 -
Endocrinology and Metabolism Clinics of... Mar 2008Acromegaly is caused by growth hormone hypersecretion, mostly from a pituitary adenoma, driving insulin-like growth factor 1 overproduction. Manifestations include... (Review)
Review
Acromegaly is caused by growth hormone hypersecretion, mostly from a pituitary adenoma, driving insulin-like growth factor 1 overproduction. Manifestations include skeletal and soft tissue growth and deformities; and cardiac, respiratory, neuromuscular, endocrine, and metabolic complications. Increased morbidity and mortality require early and tight disease control. Surgery is the treatment of choice for microadenomas and well-defined intrasellar macroadenomas. Complete resection of large and invasive macroadenomas rarely is achieved; hence, their low rate of disease remission. Pharmacologic treatments, including long-acting somatostatin analogs, dopamine agonists, and growth hormone receptor antagonists, have assumed more importance in achieving biochemical and symptomatic disease control.
Topics: Acromegaly; Dopamine; Hormone Antagonists; Human Growth Hormone; Humans; Pituitary Neoplasms
PubMed: 18226732
DOI: 10.1016/j.ecl.2007.10.002