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The discovery of RET mutations in multiple endocrine neoplasia type 2A (MEN2A) in 1993 ignited a revolution in our understanding of this versatile receptor. Since then, the influence of RET has expanded to encompass diverse organs, including the pituitary gland. This review explores the multifaceted role of RET in somatotrophs, focusing on two opposing pathways: proliferation versus differentiation and apoptosis.
View Article and Find Full Text PDFPituitary Acrogigantism: From the Past to the Future.
Front Horm Res
November 2024
Department of Endocrinology, Centre Hospitalier Universitaire de Liège, University of Liège, Liège, Belgium.
Pituitary acrogigantism is a very rare disease that is caused by chronic growth hormone (GH) axis excess that begins during childhood and adolescence. As such, it represents one of the most severe manifestations of acromegaly. In most cases, acrogigantism is caused by a pituitary adenoma, but hyperplasia can also accompany the adenoma or rarely occur alone.
View Article and Find Full Text PDFEndoscopic Transsphenoidal Surgery in Growth-Hormone Pituitary Adenomas (GH PitNETs): Current Indications, Limitations, and the Importance of a Multidisciplinary Approach.
Front Horm Res
November 2024
Neurosurgery Unit, Department of Neurosciences, Catholic University School of Medicine, Rome, Italy.
Acromegaly and gigantism are rare diseases, usually caused by a growth hormone-secreting pituitary adenoma, recently renamed GH-secreting pituitary neuroendocrine tumor (GH-PitNET). The transsphenoidal approach is the mainstay of treatment, although a non-negligible number of patients require a multimodal approach with neo-adjuvant or adjuvant medical and radiation therapy. Understanding the clinical complexity of acromegaly and gigantism is essential to improve treatment safety and success.
View Article and Find Full Text PDFGrowth hormone receptor antagonist pegvisomant and its role in the medical therapy of growth hormone excess.
Best Pract Res Clin Endocrinol Metab
July 2024
Centre for Endocrinology, Barts and The London School of Medicine, Queen Mary University of London, Charterhouse Square, London EC1M 6BQ, UK. Electronic address:
Pegvisomant is a growth-hormone (GH) receptor antagonist that prevents the formation of the active heterotrimer of the dimerised GH receptor and the GH molecule necessary for downstream signal transduction. Over the past 20 years, it has become a key therapeutic option for physicians treating syndromes of GH/IGF-1 excess. Sufficient longitudinal follow-up data suggest that it can be deemed both safe and effective.
View Article and Find Full Text PDFGermline AIP variants in sporadic young acromegaly and pituitary gigantism: clinical and genetic insights from a Han Chinese cohort.
Endocrine
September 2024
Department of Endocrinology and Metabolism, Huashan Hospital, Fudan University, No. 12 Wulumuqi Middle Road, Shanghai, China.
Purpose: Variants in the Aryl hydrocarbon receptor-interacting protein (AIP) gene have been identified in sporadic acromegaly and pituitary gigantism, especially in young patients, with a predisposition to aggressive clinical phenotype and poor treatment efficacy. The clinical characteristics of patients with sporadic acromegaly and pituitary gigantism as well as AIP variants in Han Chinese have been rarely reported. We aimed to identify AIP gene variants and analyze the clinical characteristics of patients with sporadic acromegaly and pituitary gigantism in Han Chinese.
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