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Soluble α-klotho (sαKl) is an isoform of the α-klotho protein that regulates calcium metabolism but also has anti- aging, anti-apoptotic, and tumor suppressor effects. The assumption that sαKl could inhibit the insulin-like growth factor 1 (IGF-1) signaling pathway has increased the interest of its research in acromegaly - a pathology characterized by an excess of growth hormone (GH) and IGF-1. This review aimed to identify the role of sαKl in acromegaly. Most studies have identified higher concentrations of sαKl in acromegaly versus non-GH pituitary adenomas (PAs) or healthy subjects. Also, the sαKl level was higher in active acromegaly versus controlled or cured disease. Moreover, sαKl positively correlated with GH, IGF-1, and GH-secreting PA size. The increased level of sαKl seems to be due to the autonomous secretion of GH, considering their drastic decrease after transsphenoidal surgery. The data regarding the relationship between sαKl, age, and gender in acromegaly are inconsistent. The latest research noted a correlation between sαKl and various parameters for monitoring the quality of life in acromegaly. In conclusion, sαKl could reflect the disease activity. Furthermore, sαKl would represent an alternative for monitoring acromegalic patients with discrepancies between IGF-1 and GH. [ABSTRACT FROM AUTHOR]

Pituitary adenomas are frequent benign tumors that cause a high morbidity due to their complications. Most cases are sporadic but 5% arise in a familial setting, associated with other tumors (as is for MEN1 and MEN4, Carney Complex, McCune-Albright Syndrome) or without other associated disease as is for FIPA (Familial Isolated Pituitary Adenomas) families. AIP (Aryl-hydrocarbon Receptor Interacting Protein), a tumor suppressor gene, is mutated in approximately 30% of FIPA patients and in a much lesser percent in sporadic pituitary adenomas. Patients harboring an AIP mutation have certain characteristics: are younger and with larger pituitary adenomas, usually secreting growth hormone or prolactin and more resistant to current therapies as compared to AIP negative patients. Recently a new genotype was identified in FIPA patients with early onset gigantism - microduplications on chromosome Xq26.3 and the syndrome was called X-LAG (X-linked acrogigantism). In more than a half of FIPA patients the genetic background of the disease is still unknown and more work is needed in this direction. [ABSTRACT FROM AUTHOR]

Pituitary adenomas are frequent benign tumors that cause a high morbidity due to their complications. Most cases are sporadic but 5% arise in a familial setting, associated with other tumors (as is for MEN1 and MEN4, Carney Complex, McCune-Albright Syndrome) or without other associated disease as is for FIPA (Familial Isolated Pituitary Adenomas) families. AIP (Aryl-hydrocarbon Receptor Interacting Protein), a tumor suppressor gene, is mutated in approximately 30% of FIPA patients and in a much lesser percent in sporadic pituitary adenomas. Patients harboring an AIP mutation have certain characteristics: are younger and with larger pituitary adenomas, usually secreting growth hormone or prolactin and more resistant to current therapies as compared to AIP negative patients. Recently a new genotype was identified in FIPA patients with early onset gigantism - microduplications on chromosome Xq26.3 and the syndrome was called X-LAG (X-linked acrogigantism). In more than a half of FIPA patients the genetic background of the disease is still unknown and more work is needed in this direction. [ABSTRACT FROM AUTHOR]