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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
Clinically, the features of acromegaly depend on the levels of serum IGF-1 and GH, and are contingent upon the duration of the delay in diagnosis. Typical features develop over many years and include visual disturbance due to mass effects, joint discomfort, mandibular prognathism and overbite, infertility, or menstrual irregularities; changes in hand and foot size are seen, and excessive sweating is very common. Acromegaly is associated with conditions such as type 2 diabetes, hypertension, and obstructive sleep apnea. There is a higher prevalence of colorectal neoplasms in patients with acromegaly. GH-producing tumors slowly grow and patients present usually over the age of 50 years. A common clinical manifestation is joint pain which affects around 70% at the time of diagnosis.34 In younger patients the diagnosis may be less obvious, while onset before epiphyseal fusion leads to gigantism.
The Endocrine System and Its Disorders
Published in Walter F. Stanaszek, Mary J. Stanaszek, Robert J. Holt, Steven Strauss, Understanding Medical Terms, 2020
Walter F. Stanaszek, Mary J. Stanaszek, Robert J. Holt, Steven Strauss
Pituitary hypersecretion of the growth hormone somatotropin during the period of skeletal development results in giantism, an abnormal increase in the length of skeletal structures causing unusually large body size (hence the name). Acromegaly (acro = extremity, plus megaly, large), a form of gigantism that affects adults, is often caused by a pituitary tumor that produces excess growth hormone after skeletal development is normally complete. Rather than affecting skeletal length, its effects are seen as thickening of cartilage and bone with widening of the jaw, hands, feet, eyebrow ridges, and soft tissue. Conversely, hyposecretion of somatotropin results in dwarfism, the condition seen in dwarfs and midgets. Dwarfism can also result from a deficiency in thyroid hormones.
Regulation of the Pituitary Gland by Dopamine
Published in Nira Ben-Jonathan, Dopamine, 2020
hGH is both physiologically and clinically a very important protein hormone. It reaches its highest serum levels during puberty, when it affects somatogenic growth and helps to maintain multiple tissues and organs, general health and homeostasis. A reduction in the rate of hGH production begins in middle age, with the decline continuing to old age. The full manifestation of disorders of GH release and/or action depends upon the age at which they occur. Excessive GH is almost always caused by a benign GH-producing pituitary tumor (adenoma). Children with this condition develop great stature (gigantism), whereas a prolonged excessive GH level in adults produces acromegaly, characterized by swelling of the hands and feet and altered facial features. Patients with acromegaly also have organ enlargement and serious disorders such as high blood pressure, diabetes and heart disease.
A case of neurofibromatosis type 1 and unilateral glaucoma with ectropion uveae
Published in Ophthalmic Genetics, 2022
Akshaya L. Thananjeyan, Tanya Karaconji, Maree Flaherty, Sophia Zagora, Robyn V. Jamieson, John R. B. Grigg
Our patient, on initial presentation, was conservatively managed until developing unilateral right-sided ocular hypertension. It has been previously proposed that globe enlargement in NF1 may not solely be attributed to ocular hypertension or glaucoma, as in cases with regional gigantism, ocular overgrowth can play a significant role in its manifestation (6). Hoyt et al. (6) documented a case of enlarging buphthalmos in a patient with NF1 and hemifacial hypertrophy with only moderately elevated IOP, attributing globe enlargement to additional factors including ocular gigantism and generalized hyperplasia of the orbit while drawing parallels to how regional gigantism affected surrounding structures. Similar to our patient, clinically evident buphthalmos preceded facial hemihypertrophy.
Transient Permeation Enhancer® (TPE®) technology for oral delivery of octreotide: a technological evaluation
Published in Expert Opinion on Drug Delivery, 2021
By estimating the acromegaly market at 30% of total sales for SRLs, the current worldwide sales of Sandostatin® for acromegaly can be calculated to be ~$ 429 million. Somatuline® had overall sales of 369 USD million in 2020 [18], with a proportionate allocation for acromegaly of ~178 USD million. Somavert® produced 264 USD million in sales across the acromegaly and gigantism market in 2020 [19]. The combined acromegaly and gigantism market are expected to grow at a compound annual growth rate of 4.4% to 2029, reaching a global value of 2.2 USD billion [20]. Oral octreotide will therefore target the expanding ~$800 million acromegaly market currently held by Sandostatin® and other SRLs, but in the longer term will also likely seek label extension to treat gigantism and NETs. The commercial rationale of MYCAPPSA® as a treatment for acromegaly in the first instance is clear. The vast majority of acromegaly patients are concentrated in a small number of medical centers [21], which means that patient access is streamlined. Moreover, since SRL injections are already reimbursed by US insurance providers, this is also a helpful precedent for an oral counterpart seeking insurance coverage. Chiasma has reported sales of 1.9 USD million for MYCAPPSA® in Quarter 1, 2021 [22].
Burden of acromegaly in the United States: increased health services utilization, location of care, and costs of care
Published in Journal of Medical Economics, 2021
Antonio Ribeiro-Oliveira, Richard A. Brook, Kathryn A. Munoz, Ian A. Beren, John D. Whalen, Nathan L. Kleinman, Kevin C. J. Yuen
The retrospective patient identification methodology utilized specific International Classification of Diseases (ICD) codes to highlight potential acromegaly claims. This methodology was also used in a previous study of patients with acromegaly by the authors19. Claims using ICD-9 code 253.0 (short description, acromegaly or gigantism)23 or ICD-10 code E22.0 (short description, acromegaly or pituitary gigantism)24, identified patients with acromegaly. The identified population included employees with acromegaly in addition to any identified spouses (including domestic partners) with acromegaly receiving health benefits through the employee. To be included in the study, patients (age range, 18–64 years) had to have at least 12 continuous months of eligibility for healthcare (and prescription drugs). Additionally, all eligible patients had to meet the following criteria:At least two diagnoses of acromegaly at least 30 days apart orOne diagnosis of acromegaly plus either a diagnosis of pituitary adenoma or one claim for pituitary surgery (hypophysectomy) or stereotactic radiosurgery