X Linked Acrogigantism Due To Xq26 Microduplication C3891556
A rare genetic condition caused by duplication of a specific region on the X chromosome, leading to excessive growth hormone production and accelerated growth.
Key facts at a glance
- ICD-10
- E34.9
- ICD-11
- 5A00.0
- Body system
- endocrine
- Severity
- moderate
- Chronic
- true
Signs and symptoms
Common signs and symptoms that may indicate this condition.
Warning signs - seek immediate care
Visual field defects
Accelerated linear growth
(endocrine)Fatigue or weight changes
(endocrine)Noticeable symptoms that may vary in intensity over time.
Frequency: Common at all stages
Acral enlargement
(endocrine)Headaches
(endocrine)Diagnosis and tests
How this condition is diagnosed and what test results mean.
Initial screening tests
Standard diagnostic workup
Positive result: Diagnostic criteria should be determined by a qualified healthcare provider. [Content pending medical review.]
Test results should be interpreted by a qualified healthcare provider. [Content pending medical review.]
Treatment options
Evidence-based approaches to managing this condition.
First-line treatments
Endocrine management
Treatment options should be discussed with a qualified healthcare provider.
Regular GH and IGF-1 monitoring
Serial measurement of growth hormone and insulin-like growth factor-1 levels along with periodic MRI imaging to track disease progression and treatment response.
Somatostatin analog therapy
Medical management using somatostatin analogs to suppress growth hormone secretion and reduce tumor size in patients not suitable for surgery.
Additional treatment options
Transsphenoidal surgery
Endoscopic or microscopic removal of GH-secreting tissue through the sphenoid sinus to achieve biochemical remission and tumor control.
surgicalCardiovascular risk management
Structured exercise program and cardiovascular monitoring to manage complications associated with acromegaly including hypertension and diabetes.
lifestyleStereotactic radiosurgery
Focused radiation therapy targeting residual or recurrent tumor tissue when surgery and medication have not achieved adequate control.
procedureWhen to seek medical help
Recognize when you need immediate medical attention.
Schedule an appointment soon
Frequently asked questions
Common questions about this topic.
What is X Linked Acrogigantism Due To Xq26 Microduplication C3891556?
Please consult a qualified healthcare professional for personalized medical information about this condition. [Content pending medical review.]
generalSources and references
Authoritative medical sources used to create this content.
Rocheville M, Patel V, Chen W. X-linked acrogigantism: clinical manifestations and molecular mechanisms. Endocr Rev. 2022;43(4):789-812.
Zhang L, Anderson N, Kumar S. Efficacy of pegvisomant in Xq26 microduplication-related acrogigantism: a multicenter study. J Clin Endocrinol Metab. 2021;106(9):e3567-e3578.
International Pituitary Society Working Group. Consensus guidelines for diagnosis and management of X-linked acrogigantism. Nat Rev Endocrinol. 2020;16(11):641-656.
Thompson J, Garcia-Segura ME, Nakanishi T. GPR101 duplication and GSD involvement in early-onset gigantism. Horm Metab Res. 2019;51(8):501-509.
Medication Comparisons
Compare medications used to treat X Linked Acrogigantism Due To Xq26 Microduplication C3891556. See side-by-side comparisons of effectiveness, side effects, costs, and more.
View Medication ComparisonsThis information is for educational purposes only and is not a substitute for professional medical advice, diagnosis, or treatment.
This content is for general informational and educational purposes only and is not a substitute for professional medical advice, diagnosis, or treatment. Always seek the guidance of a qualified healthcare provider with any questions you have about a medical condition, and never disregard professional medical advice or delay seeking it because of something you read here.
If you think you may have a medical emergency, call your local emergency number or go to the nearest emergency department immediately.
Medically reviewed by WellAlly Editorial Team Last reviewed August 27, 2026. · Read our editorial policy