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Clinical Cardiology Mar 2018Acromegalic cardiomyopathy is the leading cause of morbidity and all-cause mortality in patients with acromegaly. Though acromegaly is a rare condition, the associated... (Review)
Review
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. These molecular changes manifest commonly as biventricular hypertrophy, diastolic and systolic dysfunction, and valvular regurgitation. Early recognition of the condition is paramount, though the insidious progression of the disease commonly results in a late diagnosis. Biochemical testing, based on IGF-1 measurements, is the gold standard of diagnosis. Management should be centered on normalizing serum levels of both IGF-1 and GH. Transsphenoidal resection remains the most cost-effective and permanent treatment for acromegaly, though medical therapy possesses benefit for those who are not surgical candidates. Ultimately, achieving control of hormone levels results in a severe reduction in mortality rate, underscoring the importance of early recognition and treatment.
Topics: Acromegaly; Cardiomyopathies; Diagnostic Techniques, Cardiovascular; Disease Management; Global Health; Humans; Morbidity
PubMed: 29574794
DOI: 10.1002/clc.22867 -
Endocrinology and Metabolism Clinics of... Dec 2022Acromegaly has a substantial negative impact on quality of life (QoL). This review aims to discuss the impact of acromegaly on QoL from the clinical perspective as well... (Review)
Review
Acromegaly has a substantial negative impact on quality of life (QoL). This review aims to discuss the impact of acromegaly on QoL from the clinical perspective as well as from the patient perspective. Furthermore, it aims to evaluate the use of patient-reported outcome measures (PROMs) in acromegaly and how PROMs aid decision-making. The recommendations presented in this review are based on recent clinical evidence on the impact of acromegaly on QoL combined with the authors' own clinical experience treating patients with acromegaly. We recommend that a patient-centered approach should be considered in treatment decisions, integrating conventional biochemical outcomes, tumor control, comorbidities, treatment complications, and PROMs, including QoL measures. This more integrated approach seems effective in treating comorbidities and improving patient-reported outcomes and is critical, as many patients do not achieve biochemical or tumor control and comorbidities, impairment in QoL may not remit even when full biochemical control is achieved.
Topics: Acromegaly; Comorbidity; Humans; Quality of Life
PubMed: 36244688
DOI: 10.1016/j.ecl.2022.04.004 -
Journal of Endocrinological... Jun 2017In 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... (Review)
Review
BACKGROUND
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.
AIM
We here reviewed the emerging aspects of the use of PEG in clinical practice in the light of the most recent literature.
RESULTS
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.
CONCLUSIONS
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.
Topics: Acromegaly; Animals; Human Growth Hormone; Humans
PubMed: 28176221
DOI: 10.1007/s40618-017-0614-1 -
Frontiers in Endocrinology 2023Growth Hormone-secreting adenomas exhibits variable biological behavior and heterogeneous natural history, ranging from small adenomas and mild disease, to invasive and... (Review)
Review
Growth Hormone-secreting adenomas exhibits variable biological behavior and heterogeneous natural history, ranging from small adenomas and mild disease, to invasive and aggressive neoplasms with more severe clinical picture. Patients not cured or controlled after neurosurgical and first-generation somatostatin receptor ligands (SRL) therapy could require multiple surgical, medical and/or radiation treatments to achieve disease control. To date, no clinical, laboratory, histopathological, or neuroradiological markers are able to define the aggressiveness or predict the disease prognosis in patients with acromegaly. Therefore, the management of these patients requires careful evaluation of laboratory assessments, diagnostic criteria, neuroradiology examinations, and neurosurgical approaches to choose an effective and patient-tailored medical therapy. A multidisciplinary approach is particularly useful in difficult/aggressive acromegaly to schedule multimodal treatment, which includes radiation therapy, chemotherapy with temozolomide and other, recent emerging treatments. Herein, we describe the role of the different members of the multidisciplinary team according to our personal experience; a flow-chart for the therapeutic approach of difficult/aggressive acromegaly patients is proposed.
Topics: Humans; Acromegaly; Growth Hormone; Pituitary Neoplasms; Human Growth Hormone; Adenoma
PubMed: 37206441
DOI: 10.3389/fendo.2023.1123267 -
The Journal of Clinical Endocrinology... Mar 2024Acromegaly treatment has greatly evolved in recent decades, but there are still patients whose acromegaly is not controlled with currently available treatments, and...
Acromegaly treatment has greatly evolved in recent decades, but there are still patients whose acromegaly is not controlled with currently available treatments, and there is a need to improve the treatment burden. Fortunately, there are new treatments under development that may increase treatment efficacy and convenience.
Topics: Humans; Acromegaly; Octreotide; Somatostatin; Peptides, Cyclic; Insulin-Like Growth Factor I
PubMed: 37757837
DOI: 10.1210/clinem/dgad568 -
Frontiers in Endocrinology 2020Acromegaly is a disease due to chronic GH excess and a consequent rise in IGF-1 levels. This rare endocrine condition is associated with metabolic alterations such as... (Review)
Review
Acromegaly is a disease due to chronic GH excess and a consequent rise in IGF-1 levels. This rare endocrine condition is associated with metabolic alterations such as hyperglycaemia, dyslipidaemia, and systemic arterial hypertension, which, in addition to GH excess-related cardiovascular changes, play critical roles in increasing cardiovascular risk and mortality rates. Biochemical control of acromegaly, achieved by means of surgical, and/or medical treatment, positively impacts on cardiovascular risk factors and metabolic alterations, reducing overall patient mortality. However, treatment modalities of acromegaly and disease control differently impact on glucose homeostasis and lipid changes, and consequently on cardiometabolic risk. In this regard, pasireotide was shown to significantly influence glucose metabolism. This review summarizes the cardiometabolic consequences of acromegaly and its treatment, focusing on available data around the effects of medical therapy with pasireotide on factors that influence cardiometabolic risk.
Topics: Acromegaly; Adenoma; Cardiovascular Diseases; Growth Hormone-Secreting Pituitary Adenoma; Heart Disease Risk Factors; Human Growth Hormone; Humans; Insulin-Like Growth Factor I; Risk Factors; Somatostatin; Treatment Outcome
PubMed: 32117056
DOI: 10.3389/fendo.2020.00028 -
Best Practice & Research. Clinical... Apr 2019Pituitary diseases are rare conditions with severe chronic multiorgan and multisystemic morbidity requiring complex multidisciplinary treatment and usually life-long... (Review)
Review
Pituitary diseases are rare conditions with severe chronic multiorgan and multisystemic morbidity requiring complex multidisciplinary treatment and usually life-long drug treatment. Most cases are caused by functioning or non-functioning pituitary adenoma. From the patient's perspective, the burden of disease is caused by the tumour itself and associated compression symptoms, interventions, hormone excess and deficiencies, systemic manifestations of these endocrine abnormalities and general psychosocial issues that can manifest in patients with a chronic condition. In this review, patient burden is classified according to classic endocrine syndromes, with burden at diagnosis and after long-term remission, and also within the framework of value-based health care and the conceptual model of wellbeing. The recently developed patient-reported outcome measurement tool that helps to evaluate burden of patients is also discussed.
Topics: Acromegaly; Adenoma; Cost of Illness; Humans; Pituitary Neoplasms; Quality of Life
PubMed: 31405752
DOI: 10.1016/j.beem.2019.101309 -
Endocrinology and Metabolism (Seoul,... Oct 2023The clinical characteristics and prognoses of acromegaly vary among patients. Assessment of current and novel predictors can lead to multilevel categorization of... (Review)
Review
The clinical characteristics and prognoses of acromegaly vary among patients. Assessment of current and novel predictors can lead to multilevel categorization of patients, allowing integration into new clinical guidelines and a reduction in the increased morbidity and mortality associated with acromegaly. Despite advances in the diagnosis and treatment of acromegaly, its pathophysiology remains unclear. Recent advancements in multiomics technologies, including genomics, transcriptomics, proteomics, metabolomics, and radiomics, have offered new opportunities to unravel the complex pathophysiology of acromegaly. This review comprehensively explores the emerging role of multiomics approaches in elucidating the molecular landscape of acromegaly. We discuss the potential implications of multiomics data integration in the development of novel diagnostic tools, identification of therapeutic targets, and the prospects of precision medicine in acromegaly management. By integrating diverse omics datasets, these approaches can provide valuable insights into disease mechanisms, facilitate the identification of diagnostic biomarkers, and identify potential therapeutic targets for precision medicine in the management of acromegaly.
Topics: Humans; Acromegaly; Multiomics; Precision Medicine; Proteomics; Genomics
PubMed: 37828709
DOI: 10.3803/EnM.2023.1820 -
Reviews in Endocrine & Metabolic... Jun 2022Pasireotide, a novel multireceptor-targeted somatostatin receptor ligand (SRL) is characterized by a higher affinity to somatostatin receptor type 5 than type 2, unlike... (Review)
Review
Pasireotide, a novel multireceptor-targeted somatostatin receptor ligand (SRL) is characterized by a higher affinity to somatostatin receptor type 5 than type 2, unlike first-generation SRLs. Because of the broader binding profile, pasireotide has been suggested to have a greater clinical efficacy in acromegaly than first-generation SRLs and to be efficacious in Cushing's disease. The consequence of this binding profile is the increased blood glucose level in some patients. This results from the inhibition of both insulin secretion and the incretin effect and only a modest suppression of glucagon. A monthly intramuscular formulation of long-acting release pasireotide has been approved for both acromegaly and Cushing's disease treatment. This review presents data on the efficacy and safety of pasireotide treatment mostly in patients with acromegaly and Cushing's disease. Moreover, other possible therapeutic applications of pasireotide are mentioned.
Topics: Acromegaly; Humans; Ligands; Pituitary ACTH Hypersecretion; Receptors, Somatostatin; Somatostatin
PubMed: 35067849
DOI: 10.1007/s11154-022-09710-3 -
Neurology India 2017
Topics: Acromegaly; Cardiomyopathies; Humans; Neurosurgical Procedures; Sphenoid Bone; Sphenoid Sinus; Treatment Outcome
PubMed: 29133685
DOI: 10.4103/0028-3886.217988