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Endocrine and Neuroendocrine Tumors
Published in Pat Price, Karol Sikora, Treatment of Cancer, 2020
Natasha Shrikrishnapalasuriyar, P.N. Plowman, Márta Korbonits, Ashley B. Grossman
Acromegaly is a rare condition, with an annual incidence of about 3–5 cases per million.33 Its prevalence in the United Kingdom is approximately 85 per million population, although this may be a significant underestimate. There is a high mortality from acromegaly if left untreated, approximately twice the normal standardized mortality rate. Acromegaly is due to an excess of growth hormone (GH) most commonly due to a GH-secreting pituitary tumor or can be due to hypothalamic or neuroendocrine tumors which secrete GH-releasing hormone. Acromegaly can occur alongside other endocrine tumors and be part of multiple syndromes including MEN1, Carney complex, McCune–Albright, and SDHx-related pituitary adenomas. It can also present as familial isolated pituitary adenoma (FIPA).
Head and Neck
Published in Bobby Krishnachetty, Abdul Syed, Harriet Scott, Applied Anatomy for the FRCA, 2020
Bobby Krishnachetty, Abdul Syed, Harriet Scott
Acromegaly and its implications Airway – the clinical features of acromegaly lead to an increased risk of a difficult ventilation and intubation. An awake fibreoptic intubation may be necessary.Respiratory – coarsening of features leading to upper airway obstruction leads to an obstructive picture on spirometry. Also, obstructive sleep apnoea can increase the risk of difficulties with ventilation and intubation and sensitivity to opioids.Cardiovascular – myocardial hypertrophy, interstitial fibrosis, hypertension and cardiomegaly lead to ischaemic heart disease and left ventricular dysfunction.Neurological – compression of surrounding structures leading to visual disturbances, increased ICP and cavernous sinus thrombosis.Endocrine – impaired glucose tolerance and diabetes mellitus complicates more than 25% of acromegalic patients hence the need for careful glucose monitoring and management.
The endocrine system
Published in Peter Kopelman, Dame Jane Dacre, Handbook of Clinical Skills, 2019
Peter Kopelman, Dame Jane Dacre
Alteration in growth Hypopituitarism and growth hormone deficiency in children may present with short stature. In contrast, excess of growth hormone causes gigantism in children and acromegaly in adults (as it occurs after fusion of the epiphyses). The latter condition may lead to patients complaining of an increase in hand size or, more commonly, shoe size.
Specific FSTL1 polymorphism may determine the risk of cardiomyopathy in patients with acromegaly
Published in Acta Cardiologica, 2022
Suleyman Nahit Sendur, Tuncay Hazirolan, Busra Aydin, Incilay Lay, Mehmet Alikasifoglu, Tomris Erbas
The demographic and clinical variables were compared between active and controlled acromegalic individuals. There were 15 (32.5%) active patients; 6 F and 9 M with a mean age of 46.8 ± 9.3 years and 31 (67.5%) controlled patients; 23 F and 8 M with a mean age of 51.9 ± 13.1 years. The frequency of female individuals in controlled acromegaly group was higher (40% in active group, 74% in controlled group, p = 0.03). There was no difference between active and controlled acromegalic cases in terms of age, height, weight, body mass index and smoking habit. The disease duration of active and controlled acromegalic patients was similar. GH, IGF-1 and IGF-1 index were higher in active patients. Macroadenomas were more frequent in both groups. Almost all patients had a history of surgery. Surgery was combined with radiotherapy in three (20%) active patients and in seven (22.6%) controlled patients. The frequency of somatostatin receptor ligand use was higher in the controlled group (53.3% in the active group, 83.8% in the controlled group, p = 0.038). Pituitary hormone deficiency frequencies were similar as well. Except for sleep apnoea, the presence of co-morbidities was not different. The frequency of cardioactive drug use was similar (Table 1).
Biotin interference in routine IDS-iSYS immunoassays for aldosterone, renin, insulin-like growth factor 1, growth hormone and bone alkaline phosphatase
Published in Scandinavian Journal of Clinical and Laboratory Investigation, 2022
Cindy Soendersoe Knudsen, Kasper Adelborg, Esben Søndergaard, Tina Parkner
The clinical consequences of interference with the IGF-1 and GH assays are likely less, as they were present only with very high doses of biotin as those used for experimental protocols among patients with multiple sclerosis or treatment of inborn errors of biotin metabolism. However, this could be relevant for patients with kidney failure taking biotin supplement, due to a reduced renal excretion of biotin. Therefore, IGF-1 and GH measurements should be interpreted with caution in these groups of patients. Since there was negative interference with both the GH and IGF-1 assay, there is a risk of a false negative in the diagnosis of acromegaly. However, the elevation in IGF-1 (the initial test for acromegaly) is markedly higher than the magnitude of biotin interference in the vast majority of patients [25]. The risk of falsely diagnosing GH deficiency is of slightly greater concern. This could potentially lead to treatment with GH in patients without GH deficiency, with potential side effects and high cost. However, only patients with a high likelihood of disease should be evaluated for GH deficiency (e.g. hypothalamic and pituitary disease) and the diagnostic workup also includes a confirmatory stimulation test (e.g. insulin tolerance test or arginine stimulation test) [26] limiting the risk of misdiagnosis. As BAP was relatively robust for biotin interference, the clinical consequences of intake of biotin on the level of this biomarker is likely negligible.
Transient Permeation Enhancer® (TPE®) technology for oral delivery of octreotide: a technological evaluation
Published in Expert Opinion on Drug Delivery, 2021
Acromegaly is a chronic non-inherited orphan condition caused in the majority of individuals by a benign pituitary adenoma. The tumor secretes elevated plasma levels of growth hormone (GH), which in turn stimulates liver production of insulin-like growth factor-1 (IGF-1) [1]. The pathogenesis is caused by tumorigenic somatotroph cells of the pituitary gland, leading to abnormal growth of joints, internal tissues and organs, accompanied by arthritis and an increased risk of diabetes and cardiovascular disease [2,3]. Initial symptoms are enlarged facial features, accompanied by joint pain. Diagnosis is made by measuring plasma IGF-1 or by carrying out a GH suppression test in response to an oral glucose challenge, followed by imaging of the pituitary gland [4]. Worldwide estimates for patients suffering from acromegaly are 28–137 cases/million people [5].