X-linked acrogigantism (X-LAG) arises from duplications on chromosome Xq26.3 involving the gene GPR101, making it the earliest-onset form of pituitary gigantism. Affected patients typically develop signs of overgrowth — increased height and weight — in the first 12–36 months of life, with features becoming increasingly prominent over time.
This protocol addresses the situation in which extensive surgical resection (debulking) followed by second-line somatostatin analog therapy has not achieved adequate reduction or control of GH and IGF-1. Failure to reach these hormonal targets is the trigger for escalation to the next treatment step.
The primary objectives are rapid and prolonged IGF-1 control alongside blunting of height gain.
DOI: 10.1016/B978-0-12-814537-1.00002-6
Duplications on chromosome Xq26.3 involving the gene GPR101 are responsible for X-LAG, the earliest onset form of pituitary gigantism described.
Patients with X-LAG typically develop signs of overgrowth in the first 12–36 months of life, with increased height and weight becoming increasingly prominent over time.
To optimize hormonal and growth parameters, when surgery and somatostatin analogs have proven inadequate, early introduction of pegvisomant can be considered.
In X-LAG cases, addition of pegvisomant at a low dose has proven safe and effective in some individuals.
In those X-LAG patients, the addition of pegvisomant can provide rapid and prolonged IGF-1 control and blunting of height gain.
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