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The Lancet. Diabetes & Endocrinology Nov 2022Growth hormone-secreting pituitary adenomas that cause acromegaly arise as monoclonal expansions of differentiated somatotroph cells and are usually sporadic. They are... (Review)
Review
Growth hormone-secreting pituitary adenomas that cause acromegaly arise as monoclonal expansions of differentiated somatotroph cells and are usually sporadic. They are almost invariably benign, yet they can be locally invasive and show progressive growth despite treatment. Persistent excess of both growth hormone and its target hormone insulin-like growth factor 1 (IGF-1) results in a wide array of cardiovascular, respiratory, metabolic, musculoskeletal, neurological, and neoplastic comorbidities that might not be reversible with disease control. Normalisation of IGF-1 and growth hormone are the primary therapeutic aims; additional treatment goals include tumour shrinkage, relieving symptoms, managing complications, reducing excess morbidity, and improving quality of life. A multimodal approach with surgery, medical therapy, and (more rarely) radiation therapy is required to achieve these goals. In this Review, we examine the epidemiology, pathogenesis, diagnosis, complications, and treatment of acromegaly, with an emphasis on the importance of tailoring management strategies to each patient to optimise outcomes.
Topics: Humans; Acromegaly; Insulin-Like Growth Factor I; Quality of Life; Human Growth Hormone; Growth Hormone; Adenoma
PubMed: 36209758
DOI: 10.1016/S2213-8587(22)00244-3 -
Mayo Clinic Proceedings Feb 2022Acromegaly is typically caused by a growth hormone-secreting pituitary adenoma, driving excess secretion of insulin-like growth factor 1. Acromegaly may result in a... (Review)
Review
Acromegaly is typically caused by a growth hormone-secreting pituitary adenoma, driving excess secretion of insulin-like growth factor 1. Acromegaly may result in a variety of cardiovascular, respiratory, endocrine, metabolic, musculoskeletal, and neoplastic comorbidities. Early diagnosis and adequate treatment are essential to mitigate excess mortality associated with acromegaly. PubMed searches were conducted using the keywords growth hormone, acromegaly, pituitary adenoma, diagnosis, treatment, pituitary surgery, medical therapy, and radiation therapy (between 1981 and 2021). The diagnosis of acromegaly is confirmed on biochemical grounds, including elevated serum insulin-like growth factor 1 and lack of growth hormone suppression after glucose administration. Pituitary magnetic resonance imaging is advised in patients with acromegaly to identify an underlying pituitary adenoma. Transsphenoidal pituitary surgery is generally first-line therapy for patients with acromegaly. However, patients with larger and invasive tumors (macroadenomas) are often not in remission postoperatively. Medical therapies, including somatostatin receptor ligands, cabergoline, and pegvisomant, can be recommended to patients with persistent disease after surgery. Select patients may also be candidates for preoperative medical therapy. In addition, primary medical therapy has a role for patients without mass effect on the optic chiasm who are unlikely to be cured by surgery. Clinical, endocrine, imaging, histologic, and molecular markers may help predict the response to medical therapy; however, confirmation in prospective studies is needed. Radiation therapy is usually a third-line option and is increasingly administered by a variety of stereotactic techniques. An improved understanding of the pathogenesis of acromegaly may ultimately lead to the design of novel, efficacious therapies for this serious condition.
Topics: Acromegaly; Cardiovascular Diseases; Growth Hormone-Secreting Pituitary Adenoma; Hormone Antagonists; Human Growth Hormone; Humans
PubMed: 35120696
DOI: 10.1016/j.mayocp.2021.11.007 -
The Journal of Clinical Endocrinology... Apr 2020The aim of the Acromegaly Consensus Group was to revise and update the consensus on diagnosis and treatment of acromegaly comorbidities last published in 2013.
OBJECTIVE
The aim of the Acromegaly Consensus Group was to revise and update the consensus on diagnosis and treatment of acromegaly comorbidities last published in 2013.
PARTICIPANTS
The Consensus Group, convened by 11 Steering Committee members, consisted of 45 experts in the medical and surgical management of acromegaly. The authors received no corporate funding or remuneration.
EVIDENCE
This evidence-based consensus was developed using the Grading of Recommendations, Assessment, Development, and Evaluation (GRADE) system to describe both the strength of recommendations and the quality of evidence following critical discussion of the current literature on the diagnosis and treatment of acromegaly comorbidities.
CONSENSUS PROCESS
Acromegaly Consensus Group participants conducted comprehensive literature searches for English-language papers on selected topics, reviewed brief presentations on each topic, and discussed current practice and recommendations in breakout groups. Consensus recommendations were developed based on all presentations and discussions. Members of the Scientific Committee graded the quality of the supporting evidence and the consensus recommendations using the GRADE system.
CONCLUSIONS
Evidence-based approach consensus recommendations address important clinical issues regarding multidisciplinary management of acromegaly-related cardiovascular, endocrine, metabolic, and oncologic comorbidities, sleep apnea, and bone and joint disorders and their sequelae, as well as their effects on quality of life and mortality.
Topics: Acromegaly; Comorbidity; Consensus; Humans; Practice Guidelines as Topic; Quality of Life
PubMed: 31606735
DOI: 10.1210/clinem/dgz096 -
The Journal of Clinical Endocrinology... Nov 2014The aim was to formulate clinical practice guidelines for acromegaly.
OBJECTIVE
The aim was to formulate clinical practice guidelines for acromegaly.
PARTICIPANTS
The Task Force included a chair selected by the Endocrine Society Clinical Guidelines Subcommittee (CGS), five experts in the field, and a methodologist. The authors received no corporate funding or remuneration. This guideline is cosponsored by the European Society of Endocrinology.
EVIDENCE
This evidence-based guideline was developed using the Grading of Recommendations, Assessment, Development, and Evaluation (GRADE) system to describe both the strength of recommendations and the quality of evidence. The Task Force reviewed primary evidence and commissioned two additional systematic reviews.
CONSENSUS PROCESS
One group meeting, several conference calls, and e-mail communications enabled consensus. Committees and members of the Endocrine Society and the European Society of Endocrinology reviewed drafts of the guidelines.
CONCLUSIONS
Using an evidence-based approach, this acromegaly guideline addresses important clinical issues regarding the evaluation and management of acromegaly, including the appropriate biochemical assessment, a therapeutic algorithm, including use of medical monotherapy or combination therapy, and management during pregnancy.
Topics: Acromegaly; Combined Modality Therapy; Endocrinology; Evidence-Based Medicine; Female; Humans; Male; Pregnancy
PubMed: 25356808
DOI: 10.1210/jc.2014-2700 -
Endocrinology and Metabolism Clinics of... Dec 2019Hypertension is one of the most frequent complications in acromegaly, with a median frequency of 33.6% (range, 11%-54.7%). Although the pathogenesis has not been fully... (Review)
Review
Hypertension is one of the most frequent complications in acromegaly, with a median frequency of 33.6% (range, 11%-54.7%). Although the pathogenesis has not been fully elucidated, it probably results from concomitant factors leading to expansion of extracellular fluid volume, increase of peripheral vascular resistance, and development of sleep apnea syndrome. Because the effect of normalization of growth hormone and insulinlike growth factor 1 excess on blood pressure levels is unclear, an early diagnosis of hypertension and prompt antihypertensive treatment are eagerly recommended, regardless of the specific treatment of the acromegalic disease and the level of biochemical control attained.
Topics: Acromegaly; Antihypertensive Agents; Humans; Hypertension
PubMed: 31655776
DOI: 10.1016/j.ecl.2019.08.008 -
Minerva Endocrinologica Jun 2018Growth hormone (GH) and insulin-like growth factor-I (IGF-I) have pleiotropic effects on the skeleton throughout the lifespan by influencing bone formation and... (Review)
Review
Growth hormone (GH) and insulin-like growth factor-I (IGF-I) have pleiotropic effects on the skeleton throughout the lifespan by influencing bone formation and resorption. Despite these positive effects on skeletal metabolism, in presence of GH and IGF-I excess, bone turnover increases excessively leading to deterioration of bone microarchitecture and high risk of fragility fractures, thereby impairing quality of life. Coexistent hypogonadism, diabetes mellitus, hypovitaminosis D, hyperparathyroidism, and over-replacement with glucocorticoids impair bone framework, however, the effects of acromegaly on bone mineral density (BMD) are still controversial and despite normalization of bone turnover after treatment, the risk for fractures remains increased. As a matter of fact, a major clinical aspect emerging from the studies published so far is the lack of clinical-diagnostic tools able to reliably predict the appearance of fractures in patients with acromegaly occurring even in the presence of normal or low-normal BMD. New radiological software and clinical devices have been employed in acromegaly patients highlighting a significant impairment of both cortical and cancellous bone. In this article, we summarize the pathophysiology, clinical aspects and the new diagnostic tools to better understand bone impairment in acromegaly.
Topics: Absorptiometry, Photon; Acromegaly; Bone Density; Bone Remodeling; Bone and Bones; Fractures, Bone; Humans
PubMed: 28880058
DOI: 10.23736/S0391-1977.17.02733-X -
QJM : Monthly Journal of the... Jul 2017Acromegaly is a rare, chronic, progressive disease characterized by an excess secretion of growth hormone (GH) and increased circulating insulin-like growth factor 1... (Review)
Review
Acromegaly is a rare, chronic, progressive disease characterized by an excess secretion of growth hormone (GH) and increased circulating insulin-like growth factor 1 (IGF-1) concentrations. It is caused by a pituitary adenoma in the vast majority of cases. The clinical diagnosis, based on symptoms related to GH excess, is often delayed due to the insidious nature of the disease. Consequently, patients often have established systemic complications at diagnosis with increased morbidity and premature mortality. Serum IGF-1 measurement is recommended as the initial screen for patients with suspected acromegaly. The gold standard diagnostic test remains the oral glucose tolerance test with concomitant GH measurement. 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, medical therapies (such as dopamine agonists, somatostatin receptor agonists and the GH receptor antagonist pegvisomant) and radiotherapy. A multidisciplinary approach is recommended with often a requirement for combined treatment modalities. With disease control, associated morbidity and mortality can be reduced. The recently published evidence-based guidelines by the Endocrine society addressed important clinical issues regarding the evaluation and management of acromegaly. This review discusses advances in our understanding of the pathophysiology of acromegaly, diagnosis of various forms of the disease and focuses on current treatment modalities, and on future pharmacological therapies for patients with acromegaly.
Topics: Acromegaly; Combined Modality Therapy; Dopamine Agonists; Glucose Tolerance Test; Growth Hormone; Human Growth Hormone; Humans; Insulin-Like Growth Factor I; Pituitary Neoplasms
PubMed: 26873451
DOI: 10.1093/qjmed/hcw004 -
Ugeskrift For Laeger Dec 2018Acromegaly is a rare and disabling disease with a plethora of symptoms and signs attributed to sustained elevations and actions of growth hormone and insulin-like growth...
Acromegaly is a rare and disabling disease with a plethora of symptoms and signs attributed to sustained elevations and actions of growth hormone and insulin-like growth factor 1. Acromegaly is characterised by excessive somatic growth and multiple comorbidities in addition to occasional compression of the optic nerve and hypopituitarism due to the underlying adenoma. The course of the disease is insidious, and a diagnostic delay of 5-10 years is typical, and this pre-diagnostic period is also associated with increased morbidity. Effective treatment is available, once the diagnosis is established.
Topics: Acromegaly; Adenoma; Comorbidity; Delayed Diagnosis; Human Growth Hormone; Humans
PubMed: 30520722
DOI: No ID Found -
Archives of Medical Research Dec 2023Acromegaly is a chronic and rare disease. The diagnosis usually takes several years. Multiple comorbidities are associated with acromegaly. Long-term exposure to growth... (Review)
Review
Acromegaly is a chronic and rare disease. The diagnosis usually takes several years. Multiple comorbidities are associated with acromegaly. Long-term exposure to growth factors may lead to complications such as the development of benign or malignant tumors. However, the association between acromegaly and cancer remains a matter of debate due to multiple limitations in epidemiological data. There is controversy between acromegaly and mortality, but evidence shows a significant improvement in mortality rates with disease control and careful management of comorbidities. Older age, increased growth hormone levels (GH) at last follow-up, higher insulin-like growth factor-1 (IGF-1) levels at diagnosis, malignancy and radiotherapy were proposed as independent predictors of mortality. In this review we summarize the current state of knowledge in this field. Incidence of different cancer types is described. Rigorous surveillance of endocrine diseases may contribute to increased tumor detection. Personalized screening should probably be recommended.
Topics: Humans; Acromegaly; Neoplasms; Comorbidity; Insulin-Like Growth Factor I; Incidence
PubMed: 38007382
DOI: 10.1016/j.arcmed.2023.102914 -
European Journal of Endocrinology Aug 2023Acromegaly is a rare and insidious disease characterized by chronic excess growth hormone, leading to various morphological changes and systemic complications. Despite... (Review)
Review
Acromegaly is a rare and insidious disease characterized by chronic excess growth hormone, leading to various morphological changes and systemic complications. Despite its low prevalence, acromegaly poses a significant socioeconomic burden on patients and healthcare systems. This review synthesizes the current state of knowledge on the psychosocial burden, disability, impact on daily life, and cost of acromegaly disease, focusing on the quality of life, partnership, medical care and treatment afflictions, participation in daily activities, professional and leisure impairment, and cost of treatment for acromegaly and its comorbidities. It also examines management strategies, coping mechanisms, and interventions aimed at alleviating this burden. A comprehensive understanding of the extent of the socioeconomic burden in acromegaly is crucial to develop effective strategies to improve treatment and care. Further research is warranted to explore the myriad factors contributing to this burden, as well as the efficacy of interventions to alleviate it, ultimately enhancing the quality of life for patients with acromegaly.
Topics: Humans; Acromegaly; Quality of Life; Comorbidity; Socioeconomic Factors
PubMed: 37536267
DOI: 10.1093/ejendo/lvad097