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Journal of Clinical Neuroscience :... Feb 2018We investigated whether acromegaly has been diagnosed earlier at the Niigata Medical and Dental University Hospital.
PURPOSE
We investigated whether acromegaly has been diagnosed earlier at the Niigata Medical and Dental University Hospital.
METHODS
Patients with acromegaly (n = 81) who underwent their first transsphenoidal surgery from 2006 to 2015 were reviewed. Two groups were compared: those who underwent surgery between 2006 and 2010 (n = 35) and those who underwent surgery between 2011 and 2015 (n = 46). We compared clinical features and serum levels of the growth hormone (GH) and insulin-like growth factor-1 (IGF-1), hypertension (HT) and diabetes mellitus (DM) prevalence between the two groups.
RESULTS
Compared with the early group, microadenomas (<10 mm) were more prevalent in the late group (0% vs. 15.2%, p < .05). Serum IGF-1 standard deviation score (SDS) was significantly lower in the late group (8.57 ± 2.50 vs. 6.44 ± 2.30, p < .001). In both groups, mean IGF-1 SDS was significantly lower in patients without DM than in those with DM (6.9 ± 2.6 vs. 8.3 ± 2.4, p = .02). Logistic regression analysis showed that serum GH and IGF-1 levels were significantly higher in patients with DM than in those without DM.
CONCLUSION
Regarding operated cases of GH-producing pituitary adenoma, acromegaly clinical manifestations tended to be milder at diagnosis in later years of the decade, and acromegaly was diagnosed at lower IGF-1 levels and in smaller lesions. Further study is mandatory for the generalization of this trend.
Topics: Acromegaly; Adenocarcinoma; Adult; Age of Onset; Aged; Diabetes Mellitus; Female; Human Growth Hormone; Humans; Hypertension; Insulin-Like Growth Factor I; Japan; Male; Middle Aged; Neurosurgical Procedures; Pituitary Neoplasms; Prevalence; Sphenoid Bone
PubMed: 29113856
DOI: 10.1016/j.jocn.2017.10.086 -
Orphanet Journal of Rare Diseases Oct 2016Secreting pituitary adenomas that cause acromegaly and Cushing's disease, as well as prolactinomas and thyrotroph adenomas, are uncommon, usually benign, slow-growing... (Review)
Review
Secreting pituitary adenomas that cause acromegaly and Cushing's disease, as well as prolactinomas and thyrotroph adenomas, are uncommon, usually benign, slow-growing tumours. The rarity of these conditions means that their diagnosis is not familiar to most non-specialist physicians. Consequently, pituitary adenomas may be overlooked and remain untreated, and affected individuals may develop serious comorbidities that reduce their quality of life and life expectancy. Because many signs and symptoms of pituitary adenomas overlap with those of other, more common disorders, general practitioners and non-endocrinology specialists need to be aware of the "red flags" suggestive of these conditions. A long duration of active disease in patients with secreting pituitary adenomas is associated with an increased risk of comorbidities and reduced quality of life. Appropriate treatment can lead to disease remission, and, although some symptoms may persist in some patients, treatment usually reduces the incidence and severity of comorbidities and improves quality of life. Therefore, correct, early diagnosis and characterization of a pituitary adenoma is crucial for patients, to trigger timely, appropriate treatment and to optimize outcome. This article provides an overview of the epidemiology of hormonal syndromes associated with pituitary adenomas, discusses the difficulties of and considerations for their diagnosis, and reviews the comorbidities that may develop, but can be prevented, by accurate diagnosis and appropriate treatment. We hope this review will help general practitioners and non-endocrinology specialists to suspect secreting pituitary adenomas and refer patients to an endocrinologist for confirmation of the diagnosis and treatment.
Topics: Acromegaly; Adenoma; Female; Humans; Male; Pituitary ACTH Hypersecretion; Pituitary Neoplasms; Prolactinoma; Quality of Life
PubMed: 27716353
DOI: 10.1186/s13023-016-0516-x -
Endocrine Journal Nov 2022Patients with acromegaly usually present with the classical signs of acromegaly, whereas patients without the specific signs or symptoms are rarely diagnosed. This...
Patients with acromegaly usually present with the classical signs of acromegaly, whereas patients without the specific signs or symptoms are rarely diagnosed. This unique entity can be named "subclinical acromegaly". This was a retrospective study. Our study group consisted of 6 patients (4 females) with incidentally diagnosed acromegaly, most following head MRI for unrelated reasons and without the specific signs of acromegaly. Mean age at diagnosis was 48.8 ± 19.2 years. Baseline IGF-1 ranged between 1.3-2.0 × upper limit of normal (ULN). MRI depicted a pituitary microadenoma in 5 patients, and one patient presented with a 12 mm intra-sellar macroadenoma. Mean calculated SAGIT clinical score was 4.8. Three patients underwent trans-sphenoidal resection; two achieved hormonal remission and one improved but did not normalize IGF-1 following surgery. Four patients (including one following surgery) were given somatostatin analogs, and three normalized IGF-1. Several patients improved clinically following treatment, reporting improvement in snoring, hypertension, or weight loss, and pituitary adenoma decreased in size in 2 patients that responded to medical treatment. We report a series of 6 patients with very mild and subclinical acromegaly. It is uncertain whether all such patients will gain clinical benefit from treatment, but most experienced clinical improvement due to treatment.
Topics: Adult; Aged; Female; Humans; Middle Aged; Acromegaly; Human Growth Hormone; Insulin-Like Growth Factor I; Pituitary Neoplasms; Retrospective Studies; Somatostatin; Treatment Outcome; Male
PubMed: 35732439
DOI: 10.1507/endocrj.EJ22-0066 -
Medicina (Kaunas, Lithuania) Jun 2022Somatostatin receptor ligands (SRLs) represent a true milestone in the medical therapy for acromegaly. The first-generation SRLs (FG-SRLs), octreotide and lanreotide,... (Review)
Review
Somatostatin receptor ligands (SRLs) represent a true milestone in the medical therapy for acromegaly. The first-generation SRLs (FG-SRLs), octreotide and lanreotide, have demonstrated good efficacy in disease control and tumor shrinkage, and are still considered first-line medical therapies. The development of long-acting release (LAR) formulations has certainly improved the therapeutic tolerability of these drugs, although many patients still experience therapy-related burden. As such, new formulations have recently been developed to improve adherence and therapeutic efficacy and more solutions are on the way. In the case of FG-SRL-resistant disease, pasireotide, the only second generation SRL currently available, demonstrated superiority in disease control and tumor shrinkage compared to FG-SRLs. However, its use in clinical practice is still limited due to concern for impairment in glucose homeostasis. In this review, we discuss the news about the present and future role of SRLs in acromegaly, exploring the therapeutical frontiers of this drug class. Moreover, we provide practical guidance on the use of pasireotide, based on the data in the literature and our clinical experience.
Topics: Acromegaly; Humans; Ligands; Receptors, Somatostatin
PubMed: 35744057
DOI: 10.3390/medicina58060794 -
Endocrinology and Metabolism (Seoul,... Mar 2019The Korean Endocrine Society (KES) published clinical practice guidelines for the treatment of acromegaly in 2011. Since then, the number of acromegaly cases,... (Review)
Review
The Korean Endocrine Society (KES) published clinical practice guidelines for the treatment of acromegaly in 2011. Since then, the number of acromegaly cases, publications on studies addressing medical treatment of acromegaly, and demands for improvements in insurance coverage have been dramatically increasing. In 2017, the KES Committee of Health Insurance decided to publish a position statement regarding the use of somatostatin analogues in acromegaly. Accordingly, consensus opinions for the position statement were collected after intensive review of the relevant literature and discussions among experts affiliated with the KES, and the Korean Neuroendocrine Study Group. This position statement includes the characteristics, indications, dose, interval (including extended dose interval in case of lanreotide autogel), switching and preoperative use of somatostatin analogues in medical treatment of acromegaly. The recommended approach is based on the expert opinions in case of insufficient clinical evidence, and where discrepancies among the expert opinions were found, the experts voted to determine the recommended approach.
Topics: Acromegaly; Antineoplastic Agents; Antineoplastic Agents, Hormonal; Attitude; Consensus; Decision Making; Expert Testimony; Humans; Injections, Intramuscular; Insurance, Health; Neuroendocrinology; Octreotide; Peptides, Cyclic; Practice Guidelines as Topic; Preoperative Period; Republic of Korea; Somatostatin
PubMed: 30912339
DOI: 10.3803/EnM.2019.34.1.53 -
Endokrynologia Polska 2019Acromegaly is a rare, chronic condition caused by growth hormone (GH) overproduction, usually due to a benign tumour of the pituitary gland. During the disease many... (Review)
Review
Acromegaly is a rare, chronic condition caused by growth hormone (GH) overproduction, usually due to a benign tumour of the pituitary gland. During the disease many complications occur, including cardiovascular disease and changes in the musculoskeletal, respiratory, and endocrine systems. Treatment includes surgery, medical therapy, and radiation. In this paper a literature review was conducted for information related to costs of management of acromegaly and its associated comorbidities using PubMed.The majority of total costs represent pharmacological treatment, especially the most common somatostatin analogues (SSA) therapy. The average reported annual cost of SSA therapy is EUR 12,000-40,000. Surgery reduces the cost of care via the possibility of avoiding lifelong pharmacological treatment. Radiotherapy is also suggested to lower the costs of therapy because about 60% of patients eventually will not require further pharmacological treatment; however, it is connected with negative outcomes like hypopituitarism, lower quality of life, and increased mortality. Cabergoline and pegvisomant are the lowest and highest priced treatments, respectively, but the overall impact on the cost of therapy is minor due to less frequent usage of these drugs. It is hard to fully estimate the impact of comorbidities of acromegaly on financial burden because patients are treated for them many years before the diagnosis of the underlying pathology. The treatment cost of comorbidities is higher in uncontrolled patients. Life-long treatment of acromegaly and its comorbidities is very expensive. Early diagnosis and successful treatment reduce direct and indirect costs.
Topics: Acromegaly; Costs and Cost Analysis; Disease Management; Female; Humans; Male; Somatostatin
PubMed: 30843180
DOI: 10.5603/EP.a2018.0080 -
The Journal of Clinical Endocrinology... Aug 2022Diabetes is a major risk factor for cardiovascular disease and death but its effect on outcomes in acromegaly is unknown. (Observational Study)
Observational Study
CONTEXT
Diabetes is a major risk factor for cardiovascular disease and death but its effect on outcomes in acromegaly is unknown.
OBJECTIVE
This work aimed to study whether diabetes affects morbidity and mortality in patients with acromegaly.
METHODS
A nationwide (Sweden), observational, matched-cohort study was conducted. Patients diagnosed with acromegaly between 1987 and 2020 were identified in the Swedish National Patient Registry and those with concomitant type 2 diabetes in the National Diabetes Registry and Drug Registry. The risk of overall mortality, and cardiovascular mortality and morbidity were estimated using Cox regression.
RESULTS
The study included 254 patients with acromegaly and concomitant type 2 diabetes (ACRO-DM group) and 532 without diabetes (ACRO group). Mean (SD) age at baseline was 62.6 (11.4) and 60.0 (12.1) years (P = .004) and the mean (SD) duration of acromegaly was 6.8 (8.1) and 6.0 (6.2) years (P = .098) in the ACRO-DM and ACRO groups, respectively. Overall mean follow-up was 9.2 years. The unadjusted overall mortality rate per 1000 person-years was 35.1 (95% CI, 27.2-44.7) and 20.1 (95% CI, 16.5-24.3) in the respective groups. The hazard ratio (HR) for overall mortality adjusted for multiple confounders was 1.58 (95% CI, 1.12-2.23) in the ACRO-DM group compared with the ACRO group. Cardiovascular mortality (HR 2.11; 95% CI, 1.09-4.10) and morbidity (HR 1.49; 95% CI, 1.21-1.82) were also increased in the ACRO-DM group.
CONCLUSION
The presence of diabetes in patients with acromegaly was associated with increased overall mortality as well as increased cardiovascular mortality and morbidity.
Topics: Acromegaly; Cardiovascular Diseases; Cohort Studies; Diabetes Mellitus, Type 2; Humans; Morbidity
PubMed: 35779017
DOI: 10.1210/clinem/dgac400 -
Neuroendocrinology 2020Over the last 2 decades, advances in the diagnosis and management of pituitary diseases have made it possible to attain an endocrine "cure" in a large proportion of... (Review)
Review
Over the last 2 decades, advances in the diagnosis and management of pituitary diseases have made it possible to attain an endocrine "cure" in a large proportion of patients. In other words, tumors can be excised or controlled with drugs, mass effects of the lesion on surrounding structures can be solved, and pituitary deficiencies can be substituted with all relevant hormones. While this is considered a satisfactory outcome for health care providers, patients often suffer from an aftermath of prior endocrine dysfunction exposure, with irreversible effects, both physically and psychologically, which have a great impact on their everyday life. Diagnostic delay, often of several years, adds a negative impact on health perception. This affects their social, professional, and family domains and determines their future life. Understanding that this may occur is important, and health care providers should offer information to prepare the patient for this difficult journey, especially in the case of acromegaly, Cushing disease, or hypopituitarism. In order to maintain a good quality of life (QoL) in the long-term, patients need to adapt to this new situation, something that may be difficult, since they often cannot continue with all the activities and rhythm they used to do. Depression is often the consequence of maladaptation to the new situation, leading to impaired QoL.
Topics: Acromegaly; Humans; Hypopituitarism; Patient Outcome Assessment; Pituitary ACTH Hypersecretion; Quality of Life
PubMed: 32101858
DOI: 10.1159/000506809 -
Skin Research and Technology : Official... Apr 2023Although the cutaneous involvement of acromegaly has been recognized, the submacroscopical skin changes and the extent of skin thickening of patients remain unclear. (Observational Study)
Observational Study
BACKGROUND
Although the cutaneous involvement of acromegaly has been recognized, the submacroscopical skin changes and the extent of skin thickening of patients remain unclear.
OBJECTIVES
This study aimed at investigating the clinical cutaneous manifestations, dermoscopic features, and skin thickness revealed by high-frequency ultrasound (HFUS) of acromegalic patients.
METHODS
A case-control observational study was conducted. Patients with acromegaly and controls were prospectively included and received thorough cutaneous examinations to compare the macroscopical and dermoscopic features. The skin thickness measured by HFUS and its correlation with clinical data were also assessed.
RESULTS
Thirty-seven and 26 patients from acromegalic and control group were included, respectively. Clinical skin manifestations were recorded in detail. Under dermoscopy, red structureless area (91.9% vs. 65.4%, p = 0.021), perifollicular orange halo (78.4% vs. 26.9%, p = 0.005), and follicular plug (70.3% vs. 3.9%, p = 0.001) in the facial area, and perifollicular pigmentation (91.9% vs. 23.1%), broom-head hairs (83.8% vs. 3.9%), honeycomb-like pigmentation (97.3% vs. 38.46%), widened dermatoglyphics (81.1% vs. 3.9%) at the extremities (p < 0.001) were more prevalent in acromegaly. The mean skin thickness was 4.10 ± 0.48 mm for acromegaly, and 3.55 ± 0.52 mm for controls (p < 0.001) but no correlation with disease duration, adenoma size, and hormone level was found in acromegaly.
CONCLUSIONS
Submacroscopical skin changes under dermoscopy and skin thickness increase assessed by HFUS can provide clinicians with subtle evidences for early detection of acromegaly and objective parameters for accurate assessment of its skin involvement.
Topics: Humans; Acromegaly; Case-Control Studies; Dermoscopy; Ultrasonography; Skin
PubMed: 37113099
DOI: 10.1111/srt.13319 -
The Journal of Clinical Endocrinology... Nov 2023Acromegaly is a rare, chronic, debilitating disorder caused by prolonged hypersecretion of growth hormone (GH) and overproduction of insulin-like growth factor I...
CONTEXT
Acromegaly is a rare, chronic, debilitating disorder caused by prolonged hypersecretion of growth hormone (GH) and overproduction of insulin-like growth factor I (IGF-I). Medical therapies, including the somatostatin receptor ligand (SRL) pasireotide, are frequently used to restore biochemical control.
OBJECTIVE
As patients often receive therapy over prolonged periods, long-term data from real-life settings are needed.
METHODS
A retrospective analysis was performed using a prospectively maintained database of all patients with acromegaly from our primary care center who were enrolled in clinical studies with pasireotide (first visit November 2008). The main outcome measures were safety and biochemical control (age-adjusted IGF-I ≤ upper limit of normal).
RESULTS
Patients (n = 50) entered 4 parental studies and 30 continued in the rollover; at data cutoff (June 2022), 27 were still receiving pasireotide. Overall, median (range) exposure was 58 (3-137) months. Normal IGF-I was achieved in 54%, and acromegaly symptoms and quality of life were improved with treatment. No predictors of pasireotide response were identified; however, controlled patients had smaller tumors and lower GH at baseline. Tumor volume reduction occurred in 63% of evaluable patients (n = 10/16). Most patients presented hyperglycemic events, including 63.2% of patients with normal glucose before treatment. Older patients and those with higher IGF-I, glucose, and HbA1c at baseline had higher glucose and HbA1c during pasireotide treatment.
CONCLUSION
Pasireotide provided clinical benefit and was well tolerated for more than 11 years of treatment in acromegaly patients, most of whom were resistant to first-generation SRLs.
Topics: Humans; Acromegaly; Insulin-Like Growth Factor I; Glycated Hemoglobin; Retrospective Studies; Quality of Life; Treatment Outcome; Human Growth Hormone; Growth Hormone; Glucose; Adenoma
PubMed: 37357993
DOI: 10.1210/clinem/dgad378