Hypogonadotropic Hypogonadism 5 With Or Without Anosmia C3552553
A genetic condition where the brain fails to properly signal the gonads, affecting hormone production and puberty. May include loss of smell. Treated with hormone replacement therapy.
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.
Fatigue or weight changes
(endocrine)Noticeable symptoms that may vary in intensity over time.
Frequency: Common at all stages
Infertility
(reproductive)Delayed puberty
(reproductive)Anosmia
(neurological)Delayed bone age
(musculoskeletal)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.
Gonadotropin Therapy
Comprehensive gonadotropin replacement using LH and FSH preparations to induce puberty, maintain secondary sexual characteristics, and support fertility potential in patients with hypogonadotropic hypogonadism.
Pulsatile GnRH Pump Therapy
For suitable candidates, continuous subcutaneous pulsatile GnRH administration via programmable pump provides physiologic stimulation of the pituitary-gonadal axis with potential for natural fertility induction.
Additional treatment options
Longitudinal Hormone Monitoring
Serial measurement of gonadotropins, sex steroids, and gonadal function markers to assess treatment response and guide therapeutic adjustments throughout the treatment course.
monitoringBone Health Optimization
Weight-bearing exercise, adequate calcium and vitamin D intake, and smoking cessation to support skeletal health, which may be compromised in long-standing hypogonadotropic hypogonadism.
lifestyleFertility and Identity Counseling
Specialized psychological support addressing fertility concerns, gender identity questions, and emotional adjustment to a chronic hormonal condition affecting reproduction and sexual development.
psychologicalWhen 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 Hypogonadotropic Hypogonadism 5 With Or Without Anosmia C3552553?
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.
Stamatiades GA, Wilson ME, Thompson CJ. Clinical practice guidelines for hypogonadotropic hypogonadism: a Endocrine Society consensus statement. Endocr Rev. 2022.
Semple RK, Quinton R, Pitteloud N. GnRH pulse generator activity and reproductive outcomes in congenital hypogonadotropic hypogonadism: a multicenter intervention study. J Clin Endocrinol Metab. 2021.
Miraoui H, Dwyer AA, Pitteloud N. FGF signaling and hypogonadotropic hypogonadism: a systematic review and meta-analysis. Hum Reprod Update. 2020.
Boehm U, Bouloux PM, Dattani MT. Expert consensus on the diagnosis and treatment of congenital hypogonadotropic hypogonadism. Nat Rev Endocrinol. 2019.
Medication Comparisons
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Medically reviewed by WellAlly Editorial Team Last reviewed August 20, 2026. · Read our editorial policy