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Journal of Endocrinological... Feb 2016Hyperglycemia is a common feature associated with states of increased growth hormone secretion and glucocorticoid levels. The purpose of these guidelines is to assist... (Review)
Review
Hyperglycemia is a common feature associated with states of increased growth hormone secretion and glucocorticoid levels. The purpose of these guidelines is to assist clinicians and other health care providers to take evidence-based therapeutic decisions for the treatment of hyperglycemia in patients with growth hormone and corticosteroid excess. Both the SID and SIE appointed members to represent each society and to collaborate in Guidelines writing. Members were chosen for their specific knowledge in the field. Each member agreed to produce-and regularly update-conflicts of interest. The authors of these guidelines prepared their contributions following the recommendations for the development of Guidelines, using the standard classes of recommendation shown below. All members of the writing committee provided editing and systematic review of each part of the manuscript, and discussed the grading of evidence. Consensus was guided by a systematic review of all available trials and by interactive discussions.
Topics: Acromegaly; Combined Modality Therapy; Consensus; Cushing Syndrome; Diabetes Mellitus; Endocrinology; Evidence-Based Medicine; Glucocorticoids; Human Growth Hormone; Humans; Hyperglycemia; Italy; Precision Medicine; Societies, Scientific
PubMed: 26718207
DOI: 10.1007/s40618-015-0404-6 -
Nutrition, Metabolism, and... Feb 2016Hyperglycemia is a common feature associated with states of increased growth hormone secretion and glucocorticoid levels. (Review)
Review
BACKGROUND
Hyperglycemia is a common feature associated with states of increased growth hormone secretion and glucocorticoid levels.
AIMS
The purpose of these guidelines is to assist clinicians and other health care providers to take evidence-based therapeutic decisions for the treatment of hyperglycemia in patients with growth hormone and corticosteroid excess.
METHODOLOGY
Both the SID and SIE appointed members to represent each society and to collaborate in Guidelines writing. Members were chosen for their specific knowledge in the field. Each member agreed to produce--and regularly update--conflicts of interest. The Authors of these guidelines prepared their contributions following the recommendations for the development of Guidelines, using the standard classes of recommendation shown below. All members of the writing committee provided editing and systematic review of each part of the manuscript, and discussed the grading of evidence. Consensus was guided by a systematic review of all available trials and by interactive discussions.
Topics: Acromegaly; Biomarkers; Blood Glucose; Consensus; Cushing Syndrome; Endocrinology; Humans; Hyperglycemia; Hypoglycemic Agents; Italy; Societies, Medical; Treatment Outcome
PubMed: 26905474
DOI: 10.1016/j.numecd.2016.02.001 -
World Neurosurgery Jun 2018Plurihormonal adenomas (PHAs) represent 10%-15% of all functioning pituitary adenomas. The most frequent hormonal associations are with prolactin and growth hormone... (Review)
Review
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.
METHODS
We searched PubMed using the terms "plurihormonal pituitary adenoma," "ACTH GH pituitary adenoma," and "acromegaly AND Cushing's disease". In the 17 articles that were selected for literature review, only 20% (4/20) of patients presented with clinical signs of both diseases. Histologically, 19 were pituitary adenomas composed of two distinct cell populations, while only in 1 case was there evidence of a single cell producing both ACTH and GH. In the case reported here, a 60-year-old woman was incidentally diagnosed with a pituitary microadenoma. Endocrine assessment documented increased levels of insulin-like growth factor 1 and GH; ACTH and cortisol values were within normal ranges. Echocardiography documented ventricular hypertrophy. Because of clinical and biochemical evidence of acromegaly, surgery was recommended. Postoperatively, hormonal replacement therapy was started because of adrenal insufficiency. Her antihypertensive therapy was discontinued due to evidence of normal blood pressure values. Histological examination revealed an ACTH-GH PHA with 2 distinct populations of secreting cells. At 3-year follow-up, the patient showed stable clinical remission and was no longer receiving hormonal replacement therapy.
CONCLUSIONS
This is an additional case to the 20 previously reported cases of ACTH-GH PHA. Awareness of this relatively rare entity is clinically relevant. The cytogenesis of ACTH-GH PHA remains a matter of debate, and several hypotheses have been postulated.
Topics: Adenoma; Adrenocorticotropic Hormone; Antineoplastic Agents, Hormonal; Female; Growth Hormone; Humans; Middle Aged; Pituitary Neoplasms; Prolactin
PubMed: 29501516
DOI: 10.1016/j.wneu.2018.02.120 -
The prevalence of acromegaly is higher than previously reported: Changes over a three-decade period.Clinical Endocrinology Dec 2022To study time-related changes in the prevalence and patient characteristics of acromegaly, as well as to assess the impact of changes in treatment on disease control.
OBJECTIVE
To study time-related changes in the prevalence and patient characteristics of acromegaly, as well as to assess the impact of changes in treatment on disease control.
METHODS
A total of 107 patients with acromegaly were identified by healthcare registries and subsequently validated by patient chart review over a three-decade period (1992-2021). A systematic literature review focusing on the incidence and prevalence of acromegaly was performed identifying 31 studies.
RESULTS
The prevalence of acromegaly significantly increased throughout the study period (R = 0.94, p < .001) and was 122 cases/10 persons in 2021 whereas the annual incidence remained constant at 4.6 cases/10 persons. The age at the first sign of acromegaly and the age at diagnosis significantly increased during the study period, whereas growth hormone and insulin-like growth factor I decreased. Incidentalomas constituted 32% of all cases diagnosed with acromegaly in the last decade. Primary surgery was used in 93% of all cases, and repeated surgery decreased from 24% to 10% during the three decades. The use of first-generation somatostatin analogues (21%-48%) and second-line medical treatment (4%-20%) increased with a concomitant improvement of biochemical disease control (58%-91%).
CONCLUSION
The prevalence of acromegaly is higher than previously reported and the clinical presentation has shifted towards a milder phenotype. Modern treatment of acromegaly enables individualized treatment and disease control in the majority of patients.
Topics: Humans; Acromegaly; Prevalence; Adenoma; Somatostatin; Human Growth Hormone; Insulin-Like Growth Factor I
PubMed: 36163677
DOI: 10.1111/cen.14828 -
Frontiers in Endocrinology 2024Acromegaly is a rare endocrine disorder caused by hypersecretion of growth hormone (GH) from a pituitary adenoma. Elevated GH levels stimulate excess production of...
UNLABELLED
Acromegaly is a rare endocrine disorder caused by hypersecretion of growth hormone (GH) from a pituitary adenoma. Elevated GH levels stimulate excess production of insulin-like growth factor 1 (IGF-1) which leads to the insidious onset of clinical manifestations. The most common primary central nervous system (CNS) tumors, meningiomas originate from the arachnoid layer of the meninges and are typically benign and slow-growing. Meningiomas are over twice as common in women as in men, with age-adjusted incidence (per 100,000 individuals) of 10.66 and 4.75, respectively. Several reports describe co-occurrence of meningiomas and acromegaly. We aimed to determine whether patients with acromegaly are at elevated risk for meningioma. Investigation of the literature showed that co-occurrence of a pituitary adenoma and a meningioma is a rare phenomenon, and the majority of cases involve GH-secreting adenomas. To the best of our knowledge, a systematic review examining the association between meningiomas and elevated GH levels (due to GH-secreting adenomas in acromegaly or exposure to exogenous GH) has never been conducted. The nature of the observed coexistence between acromegaly and meningioma -whether it reflects causation or mere co-association -is unclear, as is the pathophysiologic etiology.
SYSTEMATIC REVIEW REGISTRATION
https://www.crd.york.ac.uk/prospero/, identifier CRD42022376998.
Topics: Humans; Meningioma; Acromegaly; Meningeal Neoplasms; Human Growth Hormone; Risk Factors; Adenoma
PubMed: 38919490
DOI: 10.3389/fendo.2024.1407615 -
Endocrine Practice : Official Journal... Mar 2016In a completed phase III study (C2305, Clinicaltrials.gov identifier: NCT00600886), the reported rate of biochemical control with octreotide long-acting release (LAR)... (Review)
Review
OBJECTIVE
In a completed phase III study (C2305, Clinicaltrials.gov identifier: NCT00600886), the reported rate of biochemical control with octreotide long-acting release (LAR) was lower than rates historically reported in patients pretreated and/or selected for response with somatostatin analogue (SSA) therapy. To assess whether lower efficacy rates of octreotide LAR in C2305 were influenced by study design, a systematic review of the literature was performed to evaluate response rates in previously published studies in acromegaly with similar design characteristics.
METHODS
PubMed was used to search for English-language clinical studies of acromegaly published through May 2014. Prospective studies of medically naïve patients (≥20) treated with SSAs for ≤12 months that reported efficacy rates using composite endpoint measures (growth hormone [GH] and insulin-like growth factor 1 [IGF-1]) were included. Two separate authors independently screened abstracts and full-length articles of each study to determine eligibility. All authors met to review and reach consensus when primary reviewers disagreed on the inclusion or exclusion of specific studies.
RESULTS
A total of 9 studies (N = 354 patients) were identified, with reported mean efficacy rates of 31% (range, 20-54%). Of note, reported mean efficacy rates were lower in studies enrolling patients naïve to any form of treatment (surgery, medical, and/or radiation) than in studies that enrolled only medically naïve patients. A limitation of this analysis was that inclusion criteria restricted the number of studies analyzed.
CONCLUSION
Interpretation of biochemical response rates with SSAs is critically dependent on the context of the study and should be evaluated across clinical trials with similar study design characteristics.
Topics: Acromegaly; Antineoplastic Agents, Hormonal; Clinical Trials as Topic; History, 21st Century; Humans; Octreotide; Peptides, Cyclic; Somatostatin; Treatment Outcome
PubMed: 26437217
DOI: 10.4158/EP15913.RA -
Scientific Reports Oct 2019Biochemical remission after transsphenoidal surgery is still unsatisfied in acromegaly patients with macroadenomas, especially with invasive macroadenomas. Concerning... (Comparative Study)
Comparative Study Meta-Analysis
Biochemical remission after transsphenoidal surgery is still unsatisfied in acromegaly patients with macroadenomas, especially with invasive macroadenomas. Concerning the impact of preoperative somatostatin analogues (SSAs) on surgical outcomes, previous studies with limited cases reported conflicting results. To assess current evidence of preoperative medical treatment, we performed a systematic review and meta-analysis of comparative studies. A literature search was conducted in Pubmed, Embase, and the Cochrane Library. Five randomized controlled trials (RCT) and seven non-RCT comparative studies were included. These studies mainly focused on pituitary macroadenomas though a small number of microadenoma cases were included. For safety, preoperative SSAs were not associated with elevated risks of postoperative complications. With respect to efficacy, the short-term cure rate was improved by preoperative SSAs, but the long-term cure rate showed no significant improvement. For invasive macroadenomas, the short-term cure rate was also improved, but the long-term results were not evaluable in clinical practice because adjuvant therapy was generally required. In conclusion, preoperative SSAs are safe in patients with acromegaly, and the favorable impact on surgical results is restricted to the short-term cure rate in macroadenomas and invasive macroadenomas. Further well-designed RCTs to examine long-term results are awaited to update the finding of this meta-analysis.
Topics: Acromegaly; Combined Modality Therapy; Growth Hormone-Secreting Pituitary Adenoma; Humans; Somatostatin; Treatment Outcome
PubMed: 31575930
DOI: 10.1038/s41598-019-50639-6 -
Neuroendocrinology 2021This study described a Chinese case of X-linked acrogigantism (X-LAG) and summarized the characteristics and treatment of all reported cases.
INTRODUCTION
This study described a Chinese case of X-linked acrogigantism (X-LAG) and summarized the characteristics and treatment of all reported cases.
METHODS
Clinical materials and biological samples from a 5-year and 2-month-old female due to "growth acceleration for 4 years" were collected. Array comparative genomic hybrid (aCGH) and further verification were performed. All X-LAG cases from the PubMed and Web of Science databases were collected and summarized with available data.
RESULTS
The patient presented accelerating growth since 1 year, and her height reached 134.6 cm (+5.24 standard deviation score [SDS]) when she was 5-year and 2-month old. She also had coarsening facial features, snoring, and acral enlargement. Growth hormone (GH) was not suppressed by the glucose-GH inhibition test, and insulin-like growth factor 1 (IGF-1) and prolactin (PRL) levels were elevated. Pituitary MRI revealed a pituitary enlargement with a maximum diameter of 22.3 mm. Octreotide imaging indicated the presence of a pituitary adenoma. The tumor shrank slightly after 3 courses of somatostatin analog but without clinical or biochemical remissions, of which the GH nadir value was 9.4 ng/mL, and IGF-1 was elevated to 749 ng/mL. Therefore, she underwent transsphenoidal surgery. Immunohistochemistry showed GH-positive and PRL-positive cells in the pituitary adenoma. Xq26.3 microduplication of the patient's germline DNA was identified by aCGH. Of all 35 reported cases, females accounted for 71.43%. There were 93.10% and 53.83% patients with hyperprolactinemia and hyperinsulinemia, respectively. Pathology showed that 75.00% of cases were adenomas. Ninety percent of cases had germline variants. The clinical and biochemical remission rates were 78.26% and 82.61%, respectively. However, the rate of complication occurrence during therapy reached 80%.
CONCLUSION
It is important to recognize the possibility of X-LAG when a child under 2-year old presents overgrowth. Early diagnosis and treatment are of great importance for better treatment efficacy and clinical outcome.
Topics: Acromegaly; Child, Preschool; China; Female; Genetic Diseases, X-Linked; Humans
PubMed: 33049741
DOI: 10.1159/000512240 -
Endocrine Connections Jul 2019Machine learning methods in sellar region diseases present a particular challenge because of the complexity and the necessity for reproducibility. This systematic review...
INTRODUCTION
Machine learning methods in sellar region diseases present a particular challenge because of the complexity and the necessity for reproducibility. This systematic review aims to compile the current literature on sellar region diseases that utilized machine learning methods and to propose a quality assessment tool and reporting checklist for future studies.
METHODS
PubMed and Web of Science were searched to identify relevant studies. The quality assessment included five categories: unmet needs, reproducibility, robustness, generalizability and clinical significance.
RESULTS
Seventeen studies were included with the diagnosis of general pituitary neoplasms, acromegaly, Cushing's disease, craniopharyngioma and growth hormone deficiency. 87.5% of the studies arbitrarily chose one or two machine learning models. One study chose ensemble models, and one study compared several models. 43.8% of studies did not provide the platform for model training, and roughly half did not offer parameters or hyperparameters. 62.5% of the studies provided a valid method to avoid over-fitting, but only five reported variations in the validation statistics. Only one study validated the algorithm in a different external database. Four studies reported how to interpret the predictors, and most studies (68.8%) suggested possible clinical applications of the developed algorithm. The workflow of a machine-learning study and the recommended reporting items were also provided based on the results.
CONCLUSIONS
Machine learning methods were used to predict diagnosis and posttreatment outcomes in sellar region diseases. Though most studies had substantial unmet need and proposed possible clinical application, replicability, robustness and generalizability were major limits in current studies.
PubMed: 31234143
DOI: 10.1530/EC-19-0156 -
BMC Endocrine Disorders Oct 2009Acromegaly, 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...
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 pound81,000/QALY and pound212,000/LYG. To reduce this to pound30K/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-effectiveness of therapies were to be applied to PEG it would be considered not to represent good value for money.
PubMed: 19814797
DOI: 10.1186/1472-6823-9-20