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hypogonadotropic hypogonadism
ICD-10 Codes
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Description
Hypogonadotropic Hypogonadism: A Condition Characterized by Low Sex Hormone Production
Hypogonadotropic hypogonadism (HH) is a medical condition where the hypothalamus or pituitary gland do not produce sufficient hormones to stimulate the testes (in males) or ovaries (in females) to produce sex hormones, such as testosterone and estrogen. This results in low levels of these hormones in the body.
Causes and Characteristics
HH is caused by a problem with either the hypothalamus or pituitary gland affecting the hypothalamic-pituitary-gonadal axis (HPG axis). The clinical characteristics of HH are androgen deficiency and a lack/delay/stop of pubertal sexual maturation. Low blood testosterone levels and low pituitary hormone levels confirm the diagnosis.
Symptoms
The symptoms of HH can vary depending on the individual, but may include:
- Low sex drive
- Erectile dysfunction in males
- Infertility or reduced fertility
- Delayed or absent puberty
- Low muscle mass and bone density
Types of Hypogonadism
There are two main types of hypogonadism: primary (hypergonadotropic) and secondary (hypogonadotropic). Primary hypogonadism is caused by a problem with the testes or ovaries, while secondary hypogonadism is caused by a problem with the hypothalamus or pituitary gland.
Treatment
The treatment for HH depends on the underlying cause of the condition. In some cases, hormone replacement therapy (HRT) may be prescribed to replace the missing hormones and alleviate symptoms. In other cases, surgery or other treatments may be necessary to address any underlying issues with the hypothalamus or pituitary gland.
References
- [1] HH is a form of hypogonadism that is due to a problem with the pituitary gland or hypothalamus (Source: #11)
- [2] The clinical syndrome of HH results from failure of episodic GnRH secretion or action, or disruption of gonadotropin secretion (Source: #14)
Additional Characteristics
- Hypogonadotropic hypogonadism
- Hormone replacement therapy (HRT)
- Delayed or absent puberty
- Low sex hormone production
- Androgen deficiency
- stop of pubertal sexual maturation
- Low blood testosterone levels
- Pituitary hormone levels confirm diagnosis
- Primary (hypergonadotropic) and secondary (hypogonadotropic) hypogonadism
- Erectile dysfunction in males
- Infertility or reduced fertility
- Low muscle mass and bone density
Signs and Symptoms
Hypogonadotropic hypogonadism (HH) is a condition characterized by low levels of sex hormones, such as testosterone in males and estrogen in females. This condition occurs when there is a problem with the pituitary gland or hypothalamus, which are responsible for regulating hormone production.
Common Signs and Symptoms:
- In children:
- Lack of growth and sexual development at the standard age for puberty (development may be very late or incomplete) [1]
- In males:
- Low levels of testosterone
- Decreased libido
- Erectile dysfunction
- Muscle loss and weakness
- Breast enlargement (gynecomastia)
- Infertility
- In females:
- Irregular menstrual cycles
- Hot flashes
- Energy and mood changes
- Osteoporosis
Other Possible Symptoms:
- Headaches or vision problems if the cause is a brain tumor [9]
- Sleep disturbances, osteoporosis, muscle atrophy, and decreased interest in sex [6]
It's essential to note that these symptoms can also be caused by other conditions, such as thyroid problems or iron deficiency. A healthcare provider will perform a physical exam and ask questions about medical history, medications, smoking history, and any previous health issues to diagnose hypogonadotropic hypogonadism.
References: [1] - Context result 1 [6] - Context result 6 [9] - Context result 9
Additional Symptoms
- Decreased libido
- Sleep disturbances
- Muscle atrophy
- Erectile dysfunction
- Osteoporosis
- Hot flashes
- Irregular menstrual cycles
- Lack of growth and sexual development at the standard age for puberty
- Low levels of testosterone
- Muscle loss and weakness
- Breast enlargement (gynecomastia)
- Energy and mood changes
- Headaches or vision problems if the cause is a brain tumor
- infertility
Diagnostic Tests
Hypogonadotropic hypogonadism is a condition characterized by low sex hormone levels due to impaired gonadal function, often resulting from pituitary or hypothalamic dysfunction. Diagnostic tests for this condition typically involve measuring hormone levels and assessing the response of the pituitary gland to stimulation.
Initial Laboratory Testing
Early morning measurement of serum testosterone, prolactin, FSH (Follicle-Stimulating Hormone), and LH (Luteinizing Hormone) levels is a crucial initial step in diagnosing hypogonadotropic hypogonadism [2]. These hormone measurements can help establish the presence of low sex hormone levels.
Additional Diagnostic Tests
Other diagnostic tests that may be performed to confirm the diagnosis include:
- Blood tests to measure hormone levels such as FSH, LH, TSH (Thyroid-Stimulating Hormone), prolactin, testosterone, and estradiol [3][6][7]
- LH response to GnRH (Gonadotropin-Releasing Hormone) stimulation test [9]
- MRI of the pituitary gland/hypothalamus to rule out any structural abnormalities [3][6][7]
Genetic Studies
In some cases, genetic studies may be performed to identify underlying genetic mutations that contribute to hypogonadotropic hypogonadism. The Invitae Hypogonadotropic Hypogonadism Panel analyzes genes associated with this condition [8].
It's essential to note that a comprehensive diagnostic evaluation should be conducted by an experienced healthcare professional, taking into account the individual's medical history and symptoms.
References: [1] - Not provided [2] - Initial laboratory testing should include early morning (8:00–10:00 AM) measurement of serum testosterone, prolactin, FSH, and LH levels. For the diagnosis of hypogonadotropic hypogonadism. [3] - Blood tests to measure hormone levels such as FSH, LH, TSH, prolactin, testosterone and estradiol · LH response to GnRH · MRI of the pituitary gland/hypothalamus [6] - Blood tests to measure hormone levels such as FSH, LH, TSH, prolactin, testosterone and estradiol · LH response to GnRH · MRI of the pituitary gland/hypothalamus [7] - Oct 3, 2024 — Blood tests to measure hormone levels such as FSH, LH, TSH, prolactin, testosterone and estradiol. LH response to GnRH · MRI of the pituitary ... [8] - The Invitae Hypogonadotropic Hypogonadism Panel analyzes genes that are associated with hypogonadotropic hypogonadism ( ... diagnostic laboratory performing full- ... [9] - by R Fraietta · 2013 · Cited by 308 — Low blood testosterone levels and low pituitary hormone levels confirm the hypogonadotropic hypogonadism diagnosis. A prolonged stimulated intravenous GnRH test ...
Additional Diagnostic Tests
- Early morning measurement of serum testosterone, prolactin, FSH (Follicle-Stimulating Hormone), and LH (Luteinizing Hormone) levels
- Blood tests to measure hormone levels such as FSH, LH, TSH (Thyroid-Stimulating Hormone), prolactin, testosterone, and estradiol
- LH response to GnRH (Gonadotropin-Releasing Hormone) stimulation test
- hypothalamus
- Genetic studies (e.g. Invitae Hypogonadotropic Hypogonadism Panel)
Treatment
Treatment Options for Hypogonadotropic Hypogonadism
Hypogonadotropic hypogonadism is a condition characterized by low levels of sex hormones due to a problem with the pituitary gland or hypothalamus. The treatment options for this condition are focused on replacing the deficient sex hormones.
- Testosterone Replacement Therapy: This is the primary treatment for hypogonadotropic hypogonadism in males. Testosterone can be administered through injections, skin patches, gels, or pellets to restore normal hormone levels.
- Human Chorionic Gonadotropin (hCG): hCG mimics the effects of luteinizing hormone (LH), stimulating testosterone production from the testes. It is often used in conjunction with testosterone replacement therapy.
- Clomiphene: This medication can stimulate testosterone production by the Leydig cells of the testes without affecting sperm counts or fertility.
Other Treatment Options
In some cases, treatment may also involve addressing underlying conditions that contribute to hypogonadotropic hypogonadism. For example:
- Pituitary Hormone Replacement: If a pituitary problem is the cause of hypogonadotropic hypogonadism, pituitary hormones can be given to help the body make more sex hormones.
- Surgery or Radiation Therapy: In cases where a pituitary tumor is present, surgery, medicine, radiation, or replacement of other hormones may be necessary.
**Medications Used to Treat Hypogonadotropic
Recommended Medications
- Testosterone Replacement Therapy
- Human Chorionic Gonadotropin (hCG)
- clomiphene
- Clomiphene
- hormone
💊 Drug information is sourced from ChEBI (Chemical Entities of Biological Interest) database. Always consult with a healthcare professional before starting any medication. Click on any medication name for detailed information.
Differential Diagnosis
Understanding Hypogonadotropic Hypogonadism
Hypogonadotropic hypogonadism (HH) is a condition characterized by low levels of sex hormones, such as testosterone and estrogen. This occurs due to a lack of stimulation from the pituitary gland, which normally regulates hormone production in the ovaries or testes.
Differential Diagnosis
The differential diagnosis for HH involves identifying the underlying cause of the condition. Some possible causes include:
- Tumors: Craniopharyngiomas and pituitary adenomas (e.g., prolactinoma, non-functioning tumor) can compress the pituitary gland, leading to reduced hormone production.
- Primary gonadal defect: Congenital or acquired defects in the ovaries or testes can result in hypergonadotropic hypogonadism, which is a different condition from HH.
- Medications and end-stage renal disease: Certain medications and end-stage renal disease can also lead to HH.
Key Factors to Consider
When diagnosing HH, it's essential to consider the following factors:
- Serum hormone levels: Low levels of follicle-stimulating hormone (FSH), luteinizing hormone (LH), and testosterone are characteristic of HH.
- MRI scans of the brain: Imaging studies can help identify any abnormalities in the pituitary gland or hypothalamus that may be contributing to the condition.
Differential Diagnosis Considerations
The differential diagnosis for HH involves distinguishing it from other conditions, such as:
- Constitutional delay of growth and puberty: This is a common cause of delayed puberty in boys.
- Hypergonadotropic hypogonadism: This condition is characterized by high levels of FSH and LH due to primary gonadal defects.
References
- [4] HH is caused by a lack of hormones that normally stimulate the ovaries or testes, including GnRH, FSH, and LH.
- [5] Diagnosis requires determining serum FSH, LH, and testosterone levels, as well as MRI scans of the brain to rule out any abnormalities in the pituitary gland or hypothalamus.
- [9] Careful history taking, including family and medication history, along with a physical examination, can guide the differential diagnosis.
Note: The numbers in square brackets refer to the context provided, which contains relevant information for answering this question.
Additional Differential Diagnoses
- Tumors
- Primary gonadal defect
- Medications and end-stage renal disease
- Constitutional delay of growth and puberty
- hypogonadism
- spermatogenic failure 21
- spermatogenic failure 2
- spermatogenic failure 18
- spermatogenic failure 20
- spermatogenic failure 3
- spermatogenic failure 8
- spermatogenic failure 19
- spermatogenic failure 12
- spermatogenic failure 7
- spermatogenic failure 17
- spermatogenic failure 22
- spermatogenic failure 14
- spermatogenic failure 23
- spermatogenic failure 13
- spermatogenic failure 5
- spermatogenic failure 1
- X-linked adrenal hypoplasia congenita
- hypogonadotropic hypogonadism 20 with or without anosmia
- hypogonadotropic hypogonadism 3 with or without anosmia
- hypogonadotropic hypogonadism 1 with or without anosmia
- spermatogenic failure 9
- spermatogenic failure 33
- spermatogenic failure 38
- spermatogenic failure 39
- spermatogenic failure 47
- spermatogenic failure 63
- Kallmann syndrome
Additional Information
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- disease_ontology
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- ORDO:478
- oboInOwl#id
- DOID:0090070
- core#notation
- DOID:0090070
- oboInOwl#hasAlternativeId
- DOID:7455
- IAO_0000115
- A hypogonadism characterized by a impaired signalling by gonadotrpin relasing hormone.
- oboInOwl#hasExactSynonym
- isolated congenital gonadotropin deficiency
- rdf-schema#label
- hypogonadotropic hypogonadism
- oboInOwl#inSubset
- http://purl.obolibrary.org/obo/doid#DO_rare_slim
- rdf-schema#subClassOf
- http://purl.obolibrary.org/obo/DOID_3614
- relatedICD
- http://example.org/icd10/E23.0
- 22-rdf-syntax-ns#type
- http://www.w3.org/2002/07/owl#Class
- rdf-schema#domain
- https://w3id.org/def/predibionto#has_symptom_4283
- RO_0004029
- http://purl.obolibrary.org/obo/DOID_0111034
- owl#annotatedSource
- t361974
- owl#someValuesFrom
- t375203
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