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, 78 (2), 131-136

X-LAG: How Did They Grow So Tall?


X-LAG: How Did They Grow So Tall?

Albert Beckers et al. Ann Endocrinol (Paris).


X-linked acrogigantism (XLAG) is a new, pediatric-onset genetic syndrome, due to Xq26.3 microduplications encompassing the GPR101 gene. XLAG has a remarkably distinct phenotype with disease onset occurring before the age of 5 in all cases described to date, which is significantly younger than in other forms of pituitary gigantism. These patients have mixed GH and prolactin positive adenomas and/or mixed-cell hyperplasia and highly elevated levels of GH/IGF-1 and prolactin. Given their particularly young age of onset, the significant GH hypersecretion can lead to a phenotype of severe gigantism with very advanced age-specific height Z-scores. If not adequately treated in childhood, this condition results in extreme final adult height. XLAG has a clinical course that is highly similar to some of the tallest people with gigantism in history.

Keywords: Familial isolated pituitary adenoma (FIPA); GPR101 gene; Gigantism; Le gigantisme; Le gène GPR101; L’acro-gigantisme lié au chromosome X (X-LAG); L’adénome hypophysaire; L’adénome hypophysaire familial isolé (FIPA); Pituitary adenoma; X-linked acrogigantism (X-LAG).

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