Hypogonadism

Hypogonadism is a condition characterized by reduced or no sex hormone production by the testes or ovaries Ovaries Ovaries are the paired gonads of the female reproductive system that contain haploid gametes known as oocytes. The ovaries are located intraperitoneally in the pelvis, just posterior to the broad ligament, and are connected to the pelvic sidewall and to the uterus by ligaments. These organs function to secrete hormones (estrogen and progesterone) and to produce the female germ cells (oocytes). Ovaries. Hypogonadism can result from primary (hypergonadotropic) or secondary (hypogonadotropic) failure. Hypergonadotropic hypogonadism results when the gonads fail, and hypogonadotropic hypogonadism results from failures in the hypothalamic–pituitary–gonadal axis. Symptoms include infertility Infertility Infertility is the inability to conceive in the context of regular intercourse. The most common causes of infertility in women are related to ovulatory dysfunction or tubal obstruction, whereas, in men, abnormal sperm is a common cause. Infertility, increased risk of osteoporosis Osteoporosis Osteoporosis refers to a decrease in bone mass and density leading to an increased number of fractures. There are 2 forms of osteoporosis: primary, which is commonly postmenopausal or senile; and secondary, which is a manifestation of immobilization, underlying medical disorders, or long-term use of certain medications. Osteoporosis, erectile dysfunction Erectile Dysfunction Erectile dysfunction (ED) is defined as the inability to achieve or maintain a penile erection, resulting in difficulty to perform penetrative sexual intercourse. Local penile factors and systemic diseases, including diabetes, cardiac disease, and neurological disorders, can cause ED. Erectile Dysfunction, decreased libido, and regression (or absence) of secondary sexual characteristics. Diagnosis is guided by the history, physical exam, measurement of hormone levels, and an evaluation for an underlying etiology. Management is with hormone replacement.

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Overview

Definition

Hypogonadism is a condition in which there is decreased sex hormone production by the testes or ovaries Ovaries Ovaries are the paired gonads of the female reproductive system that contain haploid gametes known as oocytes. The ovaries are located intraperitoneally in the pelvis, just posterior to the broad ligament, and are connected to the pelvic sidewall and to the uterus by ligaments. These organs function to secrete hormones (estrogen and progesterone) and to produce the female germ cells (oocytes). Ovaries.

Etiology

  • Hypergonadotropic hypogonadism (primary hypogonadism) in males:
    • Klinefelter syndrome Klinefelter syndrome Klinefelter syndrome is a chromosomal aneuploidy characterized by the presence of 1 or more extra X chromosomes in a male karyotype, most commonly leading to karyotype 47,XXY. Klinefelter syndrome is associated with decreased levels of testosterone and is the most common cause of congenital hypogonadism. Klinefelter Syndrome
    • Cryptorchidism Cryptorchidism Cryptorchidism is one of the most common congenital anomalies in young boys. Typically, this asymptomatic condition presents during a routine well-child examination where 1 or both testicles are not palpable in the scrotum. Cryptorchidism
    • Previous chemotherapy or radiotherapy treatment
    • Congenital bilateral anorchia
    • Testicular trauma
    • Gonadectomy
    • Defects in testicular determination: gonadal dysgenesis
    • Sertoli-cell-only syndrome
    • Luteinizing hormone (LH) resistance
    • Mumps virus Mumps virus Mumps is caused by a single-stranded, linear, negative-sense RNA virus of the family Paramyxoviridae and the subfamily Rublavirinae. The mumps virus is contagious and spreads only among humans by respiratory droplets or direct contact transmission from an infected person or fomite. Mumps Virus/Mumps
    • Disorders of androgen synthesis:
      • 17β-hydroxylase dehydrogenase deficiency
      • 5α-reductase deficiency
      • 17-hydroxylase deficiency
  • Hypergonadotropic hypogonadism (primary hypogonadism) in females:
    • Turner syndrome Turner syndrome Turner syndrome is a genetic condition affecting women, in which 1 X chromosome is partly or completely missing. The classic result is the karyotype 45,XO with a female phenotype. Turner syndrome is associated with decreased sex hormone levels and is the most common cause of primary amenorrhea. Turner Syndrome
    • XX and XY gonadal dysgenesis
    • Premature ovarian insufficiency
    • Autoimmune oophoritis
    • Galactosemia Galactosemia Galactosemia is a disorder caused by defects in galactose metabolism. Galactosemia is an inherited, autosomal-recessive condition, which results in inadequate galactose processing and high blood levels of monosaccharide. The rare disorder often presents in infants with symptoms of lethargy, nausea, vomiting, diarrhea, and jaundice. Galactosemia
    • Follicle-stimulating hormone (FSH) receptor gene mutations
    • LH or hCG resistance
    • Noonan syndrome
    • Previous chemotherapy or radiotherapy treatment
  • Hypogonadotropic hypogonadism (secondary hypogonadism): disruption of the hypothalamus Hypothalamus The hypothalamus is a collection of various nuclei within the diencephalon in the center of the brain. The hypothalamus plays a vital role in endocrine regulation as the primary regulator of the pituitary gland, and it is the major point of integration between the central nervous and endocrine systems. Hypothalamus or pituitary gland Pituitary gland The pituitary gland, also known as the hypophysis, is considered the "master endocrine gland" because it releases hormones that regulate the activity of multiple major endocrine organs in the body. The gland sits on the sella turcica, just below the hypothalamus, which is the primary regulator of the pituitary gland. Pituitary Gland hormonal axis
    • Sarcoidosis Sarcoidosis Sarcoidosis is a multisystem inflammatory disease that causes noncaseating granulomas. The exact etiology is unknown. Sarcoidosis usually affects the lungs and thoracic lymph nodes, but it can also affect almost every system in the body, including the skin, heart, and eyes, most commonly. Sarcoidosis
    • Hemochromatosis
    • Tuberculosis Tuberculosis Tuberculosis (TB) is an infectious disease caused by Mycobacterium tuberculosis complex bacteria. The bacteria usually attack the lungs but can also damage other parts of the body. Approximately 30% of people around the world are infected with this pathogen, with the majority harboring a latent infection. Tuberculosis spreads through the air when a person with active pulmonary infection coughs or sneezes. Tuberculosis
    • Medications:
      • Opioid analgesics Opioid analgesics Opiates are drugs that are derived from the sap of the opium poppy. Opiates have been used since antiquity for the relief of acute severe pain. Opioids are synthetic opiates with properties that are substantially similar to those of opiates. Opioid Analgesics
      • Glucocorticoids Glucocorticoids Glucocorticoids are a class within the corticosteroid family. Glucocorticoids are chemically and functionally similar to endogenous cortisol. There are a wide array of indications, which primarily benefit from the antiinflammatory and immunosuppressive effects of this class of drugs. Glucocorticoids
      • Leuprolide
    • Hypothalamic or pituitary tumors ( pituitary adenoma Pituitary adenoma Pituitary adenomas are tumors that develop within the anterior lobe of the pituitary gland. Non-functioning or non-secretory adenomas do not secrete hormones but can compress surrounding pituitary tissue, leading to hypopituitarism. Secretory adenomas secrete various hormones depending on the cell type from which they evolved, leading to hyperpituitarism. Pituitary Adenomas)
    • Congenital disorders:
      • Kallmann syndrome Kallmann syndrome Kallmann syndrome (KS), also called olfacto-genital syndrome, is a genetic condition that causes hypogonadotropic hypogonadism due to decreased secretion of gonadotropin-releasing hormone (GnRH) by the hypothalamus. The lack of sex hormones results in impaired pubertal development. Kallmann Syndrome
      • Prader-Willi syndrome Prader-Willi syndrome Prader-Willi syndrome (PWS) is a rare autosomal neurodevelopmental genetic disorders mapped to a specific region of chromosome 15 attributed to genomic imprinting. A paternally derived chromosome 15 with this deletion results in 15q11-13 paternal deletion syndrome, or PWS. Prader-Willi Syndrome and Angelman Syndrome 
      • Angelman syndrome Angelman syndrome Angelman syndrome (AS) is a rare autosomal neurodevelopmental genetic disorders mapped to a specific region of chromosome 15 attributed to genomic imprinting. A maternally derived chromosome 15 with this deletion results in 15q11-13 maternal deletion syndrome, or AS. Prader-Willi Syndrome and Angelman Syndrome
      • Gaucher disease Gaucher disease Gaucher Disease (GD) is an autosomal recessive lysosomal storage disorder caused by a deficiency of glucocerebrosidase enzyme activity, resulting in accumulation of glucocerebroside in cells and certain organs. The disease is categorized into 3 types with variable clinical presentation. Gaucher Disease
    • Other disorders:
      • Eating disorders
      • Exercise-induced hypogonadism
      • Hyperprolactinemia Hyperprolactinemia Hyperprolactinemia is defined as a condition of elevated levels of prolactin (PRL) hormone in the blood. The PRL hormone is secreted by the anterior pituitary gland and is responsible for breast development and lactation. The most common cause is PRL-secreting pituitary adenomas (prolactinomas). Hyperprolactinemia
      • Cushing syndrome
      • HIV/AIDS
      • Morbid obesity Obesity Obesity is a condition associated with excess body weight, specifically with the deposition of excessive adipose tissue. Obesity is considered a global epidemic. Major influences come from the western diet and sedentary lifestyles, but the exact mechanisms likely include a mixture of genetic and environmental factors. Obesity
      • Type 2 diabetes mellitus Diabetes mellitus Diabetes mellitus (DM) is a metabolic disease characterized by hyperglycemia and dysfunction of the regulation of glucose metabolism by insulin. Type 1 DM is diagnosed mostly in children and young adults as the result of autoimmune destruction of β cells in the pancreas and the resulting lack of insulin. Type 2 DM has a significant association with obesity and is characterized by insulin resistance. Diabetes Mellitus

Epidemiology

  • Hypogonadism may occur at any age.
  • Often underreported
  • Primary hypogonadism:
    • Most common cause in women is Turner syndrome Turner syndrome Turner syndrome is a genetic condition affecting women, in which 1 X chromosome is partly or completely missing. The classic result is the karyotype 45,XO with a female phenotype. Turner syndrome is associated with decreased sex hormone levels and is the most common cause of primary amenorrhea. Turner Syndrome (incidence: 1 in 2000–2500 live births).
    • Most common cause in men is Klinefelter syndrome Klinefelter syndrome Klinefelter syndrome is a chromosomal aneuploidy characterized by the presence of 1 or more extra X chromosomes in a male karyotype, most commonly leading to karyotype 47,XXY. Klinefelter syndrome is associated with decreased levels of testosterone and is the most common cause of congenital hypogonadism. Klinefelter Syndrome (incidence: 1 in 600 live male births).
    • Men > women (because incidence of Klinefelter syndrome Klinefelter syndrome Klinefelter syndrome is a chromosomal aneuploidy characterized by the presence of 1 or more extra X chromosomes in a male karyotype, most commonly leading to karyotype 47,XXY. Klinefelter syndrome is associated with decreased levels of testosterone and is the most common cause of congenital hypogonadism. Klinefelter Syndrome > incidence of Turner syndrome Turner syndrome Turner syndrome is a genetic condition affecting women, in which 1 X chromosome is partly or completely missing. The classic result is the karyotype 45,XO with a female phenotype. Turner syndrome is associated with decreased sex hormone levels and is the most common cause of primary amenorrhea. Turner Syndrome)
  • Secondary hypogonadism:
    • Less common than primary hypogonadism
    • Incidence: 1 in 10,000–86,000 people
    • Men = women
    • Kallmann syndrome Kallmann syndrome Kallmann syndrome (KS), also called olfacto-genital syndrome, is a genetic condition that causes hypogonadotropic hypogonadism due to decreased secretion of gonadotropin-releasing hormone (GnRH) by the hypothalamus. The lack of sex hormones results in impaired pubertal development. Kallmann Syndrome causes approximately ⅔ of congenital cases.

Pathophysiology

Normal physiology

  • The gonads function as part of the hypothalamic–pituitary–gonadal axis, which functions as a feedback loop:
    • A hypothalamic pulse generator in the arcuate nucleus → releases gonadotropin-releasing hormone (GnRH) into the hypothalamic–pituitary portal system 
    • Anterior pituitary secretes FSH and LH → stimulates gonadal activity → sex hormone production (e.g., testosterone, estrogen)
    • Gonadal hormones Gonadal hormones The gonadal hormones are produced by the human gonads: the testes and the ovaries. The primary hormones produced by these organs include androgens, estrogens, and progestins. Testosterone is the primary androgen, and estradiol and progesterone are the primary female hormones. Gonadal Hormones → ↓ FSH and LH secretion at the pituitary level → completes a feedback loop
  • In men (testes): 
    • LH → Leydig cells secrete testosterone.
    • FSH → tubular growth
  • In women ( ovaries Ovaries Ovaries are the paired gonads of the female reproductive system that contain haploid gametes known as oocytes. The ovaries are located intraperitoneally in the pelvis, just posterior to the broad ligament, and are connected to the pelvic sidewall and to the uterus by ligaments. These organs function to secrete hormones (estrogen and progesterone) and to produce the female germ cells (oocytes). Ovaries):
    • LH → theca and interstitial cells → produce progestins and androgens Androgens Androgens are naturally occurring steroid hormones responsible for development and maintenance of the male sex characteristics, including penile, scrotal, and clitoral growth, development of sexual hair, deepening of the voice, and musculoskeletal growth. Androgens and Antiandrogens
    • FSH → granulosa cells → precursor steroids to estrogen
Summary of the hypothalamic–pituitary–ovarian axis

Summary of the hypothalamic–pituitary–ovarian axis:
The hypothalamus Hypothalamus The hypothalamus is a collection of various nuclei within the diencephalon in the center of the brain. The hypothalamus plays a vital role in endocrine regulation as the primary regulator of the pituitary gland, and it is the major point of integration between the central nervous and endocrine systems. Hypothalamus secretes gonadotropin-releasing hormone (GnRH), which stimulates the anterior pituitary to release the gonadotropins follicle-stimulating hormone (FSH) and luteinizing hormone (LH). The gonadotropins then stimulate the ovary to produce estrogen and progesterone, which in turn lead to endometrial growth and maturation. Any disruption in this pathway could lead to amenorrhea.

Image by Lecturio. License: CC BY-NC-SA 4.0

Hypogonadism

Hypogonadism occurs if the hypothalamic–pituitary–gonadal axis is interrupted at any level. 

  • Primary (hypergonadotropic hypogonadism):
    • The gonad is not producing an amount of sex steroid sufficient to suppress LH and FSH.
    • Hormone levels: ↑ LH and FSH (due to lack of feedback), ↓ sex hormone 
  • Secondary (hypogonadotropic hypogonadism): 
    • Occurs from either:
      • Failure of the hypothalamic GnRH pulse generator 
      • Inability of the pituitary to respond by secreting LH and FSH
    • Hormone levels: ↓ GnRH, LH, FSH, and sex hormone
    • Most commonly from malformations in pituitary development or lesions of the pituitary that are acquired.
Pathogenesis of primary versus secondary hypogonadism

Pathogenesis of primary versus secondary hypogonadism:
Primary hypogonadism results from an issue with the testes (or ovaries Ovaries Ovaries are the paired gonads of the female reproductive system that contain haploid gametes known as oocytes. The ovaries are located intraperitoneally in the pelvis, just posterior to the broad ligament, and are connected to the pelvic sidewall and to the uterus by ligaments. These organs function to secrete hormones (estrogen and progesterone) and to produce the female germ cells (oocytes). Ovaries), resulting in decreased sex hormone despite adequate (or high) levels of gonadotropin-releasing hormone (GnRH), luteinizing hormone (LH), and follicle-stimulating hormone (FSH).
In secondary hypogonadism, an issue in the hypothalamus Hypothalamus The hypothalamus is a collection of various nuclei within the diencephalon in the center of the brain. The hypothalamus plays a vital role in endocrine regulation as the primary regulator of the pituitary gland, and it is the major point of integration between the central nervous and endocrine systems. Hypothalamus or pituitary gland Pituitary gland The pituitary gland, also known as the hypophysis, is considered the "master endocrine gland" because it releases hormones that regulate the activity of multiple major endocrine organs in the body. The gland sits on the sella turcica, just below the hypothalamus, which is the primary regulator of the pituitary gland. Pituitary Gland results in decreased GnRH, LH, and FSH, resulting in decreased sex hormone production (despite normal testes or ovaries Ovaries Ovaries are the paired gonads of the female reproductive system that contain haploid gametes known as oocytes. The ovaries are located intraperitoneally in the pelvis, just posterior to the broad ligament, and are connected to the pelvic sidewall and to the uterus by ligaments. These organs function to secrete hormones (estrogen and progesterone) and to produce the female germ cells (oocytes). Ovaries).

Image by Lecturio. License: CC BY-NC-SA 4.0

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Clinical Presentation

Clinical presentation will vary depending on the age at onset and sex.

Presentation in men

  • Prepubertal men:
    • Eunuchoidism
    • Sparse body hair
    • Absence or regression of secondary sexual characteristics
    • Voice does not deepen
    • Poor development of skeletal muscle mass
    • Delay in epiphyseal closure → long arms and legs
  • Postpubertal men:
    • Lack of energy
    • ↓ Libido
    • Erectile dysfunction
    • Depression
    • ↓ Muscle mass
    • ↓ Body hair
    • ↓ Bone mass
    • Gynecomastia Gynecomastia Gynecomastia is a benign proliferation of male breast glandular ductal tissue, usually bilateral, caused by increased estrogen activity, decreased testosterone activity, or medications. The condition is common and physiological in neonates, adolescent boys, and elderly men. Gynecomastia (more likely in primary hypogonadism)
    • Infertility (↓ sperm count)

Presentation in women

  • Prepubertal women:
    • Failure to progress through puberty Puberty Puberty is a complex series of physical, psychosocial, and cognitive transitions usually experienced by adolescents (11-19 years of age). Puberty is marked by a growth in stature and the development of secondary sexual characteristics, achievement of fertility, and changes in most body systems. Puberty
    • Absence or regression of secondary sexual characteristics
    • Primary amenorrhea Primary Amenorrhea Primary amenorrhea is defined as the absence of menstruation in a girl by age 13 years in the absence of secondary sex characteristics or by the age of 15 years with the presence of secondary sex characteristics. Etiologies can originate in the hypothalamic-pituitary-ovarian (HPO) axis or from anatomic abnormalities in the uterus or vagina. Primary Amenorrhea
  • Postpubertal women:
    • Secondary amenorrhea Secondary Amenorrhea Secondary amenorrhea is defined as the absence of menses for 3 months in a woman with previously regular menstrual cycles or for 6 months in a woman with previously irregular cycles. Etiologies involve either disruptions to the hypothalamic-pituitary-ovarian (HPO) axis or acquired obstructions in the uterus or outflow tract. Secondary Amenorrhea
    • Infertility
    • Fatigue
    • ↓ Libido

Diagnosis and Management

Laboratory evaluation

Men:

  • Initial testing:
    • ↓ Serum testosterone: 
      • Optimal timing: morning (due to diurnal variation)
      • If abnormal, should be remeasured on 1–2 separate occasions.
    • FSH levels and LH levels allow differentiation between primary and secondary hypogonadism:
      • Increased: primary hypogonadism
      • Decreased: secondary hypogonadism
  • If concerned for primary hypogonadism:
    • Is the etiology known (e.g., chemotherapy, history of mumps orchitis Orchitis Epididymitis and orchitis are characterized by acute inflammation of the epididymis and the testicle, respectively, due to viral or bacterial infections. Patients typically present with gradually worsening testicular pain and scrotal swelling along with systemic symptoms such as fever, depending on severity. Epididymitis and Orchitis)?
    • If not → karyotype analysis
  • If concerned for secondary hypogonadism:
    • Prolactin
    • Thyroid function
    • Morning cortisol
    • Iron and ferritin levels
    • Genetic testing
  • Fertility evaluation: semen analysis

Women:

  • Always start with a urine pregnancy Pregnancy Pregnancy is the time period between fertilization of an oocyte and delivery of a fetus approximately 9 months later. The 1st sign of pregnancy is typically a missed menstrual period, after which, pregnancy should be confirmed clinically based on a positive β-hCG test (typically a qualitative urine test) and pelvic ultrasound. Pregnancy: Diagnosis, Maternal Physiology, and Routine Care test or qualitative hCG test to rule out pregnancy Pregnancy Pregnancy is the time period between fertilization of an oocyte and delivery of a fetus approximately 9 months later. The 1st sign of pregnancy is typically a missed menstrual period, after which, pregnancy should be confirmed clinically based on a positive β-hCG test (typically a qualitative urine test) and pelvic ultrasound. Pregnancy: Diagnosis, Maternal Physiology, and Routine Care.
  • FSH, LH, and estradiol:
    • ↑ FSH and LH, ↓ estradiol: primary hypogonadism
    • ↓ FSH and LH, ↓ estradiol: secondary hypogonadism
  • The evaluation for an etiology is similar to the workup in men.

Imaging

  • MRI of the brain should be considered for: 
    • Pituitary hormone abnormalities (e.g., hyperprolactinemia)
    • Visual field defects
    • Neurologic abnormalities
  • MRI of the pelvis Pelvis The pelvis consists of the bony pelvic girdle, the muscular and ligamentous pelvic floor, and the pelvic cavity, which contains viscera, vessels, and multiple nerves and muscles. The pelvic girdle, composed of 2 "hip" bones and the sacrum, is a ring-like bony structure of the axial skeleton that links the vertebral column with the lower extremities. Pelvis or ultrasound of the ovaries Ovaries Ovaries are the paired gonads of the female reproductive system that contain haploid gametes known as oocytes. The ovaries are located intraperitoneally in the pelvis, just posterior to the broad ligament, and are connected to the pelvic sidewall and to the uterus by ligaments. These organs function to secrete hormones (estrogen and progesterone) and to produce the female germ cells (oocytes). Ovaries in females with concern for agenesis 
  • Ultrasonography of testes for: 
    • Suspected history of mumps orchitis Orchitis Epididymitis and orchitis are characterized by acute inflammation of the epididymis and the testicle, respectively, due to viral or bacterial infections. Patients typically present with gradually worsening testicular pain and scrotal swelling along with systemic symptoms such as fever, depending on severity. Epididymitis and Orchitis 
    • Abnormal development of the testes

Management

  • Underlying cause must be treated.
  • Hormonal replacement therapy with the goals of:
    • Promoting development and maintaining secondary sexual characteristics with normal sexual function
    • Building and sustaining normal bone Bone Bone is a compact type of hardened connective tissue composed of bone cells, membranes, an extracellular mineralized matrix, and central bone marrow. The 2 primary types of bone are compact and spongy. Structure of Bones and muscle mass
    • Aid in proper psychosocial adjustment
  • In men: testosterone replacement
  • In women: estrogen replacement
  • Pulsatile GnRH therapy aids fertility in hypogonadotropic hypogonadism.

Clinical Relevance

  • Klinefelter syndrome Klinefelter syndrome Klinefelter syndrome is a chromosomal aneuploidy characterized by the presence of 1 or more extra X chromosomes in a male karyotype, most commonly leading to karyotype 47,XXY. Klinefelter syndrome is associated with decreased levels of testosterone and is the most common cause of congenital hypogonadism. Klinefelter Syndrome: chromosomal aneuploidy characterized by the presence of ≥ 1 extra X chromosome in a male karyotype, most commonly leading to the karyotype 47, XXY. Patients present as tall, phenotypic men, with small testes, decreased body hair, gynecomastia, and infertility Infertility Infertility is the inability to conceive in the context of regular intercourse. The most common causes of infertility in women are related to ovulatory dysfunction or tubal obstruction, whereas, in men, abnormal sperm is a common cause. Infertility. Treatment consists of lifelong testosterone replacement therapy.
  • Cryptorchidism Cryptorchidism Cryptorchidism is one of the most common congenital anomalies in young boys. Typically, this asymptomatic condition presents during a routine well-child examination where 1 or both testicles are not palpable in the scrotum. Cryptorchidism: among the most common congenital anomalies in young boys. Typically, this asymptomatic condition presents during a routine well-child examination, where 1 or both testicles Testicles The testicles, also known as the testes or the male gonads, are a pair of egg-shaped glands suspended within the scrotum. The testicles have multiple layers: an outer tunica vaginalis, an intermediate tunica albuginea, and an innermost tunica vasculosa. The testicles are composed of testicular lobules and seminiferous tubules. Testicles are not palpable in the scrotum. If the testis is palpable, but not in the dependent intrascrotal location, management consists of an orchiopexy (bringing the testicle to the scrotum).
  • Pituitary adenomas Pituitary adenomas Pituitary adenomas are tumors that develop within the anterior lobe of the pituitary gland. Non-functioning or non-secretory adenomas do not secrete hormones but can compress surrounding pituitary tissue, leading to hypopituitarism. Secretory adenomas secrete various hormones depending on the cell type from which they evolved, leading to hyperpituitarism. Pituitary Adenomas: tumors that develop within the anterior lobe of the pituitary gland Pituitary gland The pituitary gland, also known as the hypophysis, is considered the "master endocrine gland" because it releases hormones that regulate the activity of multiple major endocrine organs in the body. The gland sits on the sella turcica, just below the hypothalamus, which is the primary regulator of the pituitary gland. Pituitary Gland. Nonfunctioning or nonsecretory adenomas do not secrete hormones Hormones Hormones are messenger molecules that are synthesized in one part of the body and move through the bloodstream to exert specific regulatory effects on another part of the body. Hormones play critical roles in coordinating cellular activities throughout the body in response to the constant changes in both the internal and external environments. Hormones: Overview, but compress surrounding pituitary tissue, leading to hypopituitarism Hypopituitarism Hypopituitarism is a condition characterized by pituitary hormone deficiency. This condition primarily results from a disease of the pituitary gland, but it may arise from hypothalamic dysfunction. Pituitary tumors are one of the most common causes. The majority of cases affect the anterior pituitary lobe (adenohypophysis), which accounts for 80% of the gland. Hypopituitarism. Secretory adenomas secrete various hormones Hormones Hormones are messenger molecules that are synthesized in one part of the body and move through the bloodstream to exert specific regulatory effects on another part of the body. Hormones play critical roles in coordinating cellular activities throughout the body in response to the constant changes in both the internal and external environments. Hormones: Overview depending on the cell type they evolved from, leading to hyperpituitarism. Diagnosis is confirmed with imaging. Management depends on the type of adenoma, but can include surgical resection, radiation, and dopamine agonists.
  • Mumps virus Mumps virus Mumps is caused by a single-stranded, linear, negative-sense RNA virus of the family Paramyxoviridae and the subfamily Rublavirinae. The mumps virus is contagious and spreads only among humans by respiratory droplets or direct contact transmission from an infected person or fomite. Mumps Virus/Mumps: caused by a single-stranded, linear, negative-sense RNA RNA Ribonucleic acid (RNA), like deoxyribonucleic acid (DNA), is a polymer of nucleotides that is essential to cellular protein synthesis. Unlike DNA, RNA is a single-stranded structure containing the sugar moiety ribose (instead of deoxyribose) and the base uracil (instead of thymine). RNA generally carries out the instructions encoded in the DNA but also executes diverse non-coding functions. RNA Types and Structure virus Virus Viruses are infectious, obligate intracellular parasites composed of a nucleic acid core surrounded by a protein capsid. Viruses can be either naked (non-enveloped) or enveloped. The classification of viruses is complex and based on many factors, including type and structure of the nucleoid and capsid, the presence of an envelope, the replication cycle, and the host range. Virology: Overview of the family Paramyxoviridae. Mumps Mumps Mumps is caused by a single-stranded, linear, negative-sense RNA virus of the family Paramyxoviridae. Mumps is typically a disease of childhood, which manifests initially with fever, muscle pain, headache, poor appetite, and a general feeling of malaise, and is classically followed by parotitis. Mumps Virus/Mumps manifests initially with fever Fever Fever is defined as a measured body temperature of at least 38°C (100.4°F). Fever is caused by circulating endogenous and/or exogenous pyrogens that increase levels of prostaglandin E2 in the hypothalamus. Fever is commonly associated with chills, rigors, sweating, and flushing of the skin. Fever, muscle pain Pain Pain has accompanied humans since they first existed, first lamented as the curse of existence and later understood as an adaptive mechanism that ensures survival. Pain is the most common symptomatic complaint and the main reason why people seek medical care. Physiology of Pain, headache, poor appetite, feeling generally unwell, and parotitis. Complications include meningitis Meningitis Meningitis is inflammation of the meninges, the protective membranes of the brain, and spinal cord. The causes of meningitis are varied, with the most common being bacterial or viral infection. The classic presentation of meningitis is a triad of fever, altered mental status, and nuchal rigidity. Meningitis, pancreatitis, deafness, and testicular inflammation Inflammation Inflammation is a complex set of responses to infection and injury involving leukocytes as the principal cellular mediators in the body's defense against pathogenic organisms. Inflammation is also seen as a response to tissue injury in the process of wound healing. The 5 cardinal signs of inflammation are pain, heat, redness, swelling, and loss of function. Inflammation, which can result in infertility Infertility Infertility is the inability to conceive in the context of regular intercourse. The most common causes of infertility in women are related to ovulatory dysfunction or tubal obstruction, whereas, in men, abnormal sperm is a common cause. Infertility. The infection is managed with supportive care and is preventable by vaccination Vaccination Vaccination is the administration of a substance to induce the immune system to develop protection against a disease. Unlike passive immunization, which involves the administration of pre-performed antibodies, active immunization constitutes the administration of a vaccine to stimulate the body to produce its own antibodies. Vaccination.
  • Turner syndrome Turner syndrome Turner syndrome is a genetic condition affecting women, in which 1 X chromosome is partly or completely missing. The classic result is the karyotype 45,XO with a female phenotype. Turner syndrome is associated with decreased sex hormone levels and is the most common cause of primary amenorrhea. Turner Syndrome: genetic condition affecting women, in which an X chromosome is partly or completely missing. The classic result is the karyotype 45,X0 with a female phenotype. Characteristic appearance is that of short stature, webbed neck, broad chest, widely spaced nipples, amenorrhea, and peripheral edema Edema Edema is a condition in which excess serous fluid accumulates in the body cavity or interstitial space of connective tissues. Edema is a symptom observed in several medical conditions. It can be categorized into 2 types, namely, peripheral (in the extremities) and internal (in an organ or body cavity). Edema of the hands and feet. Genetic testing confirms diagnosis. Treatment consists of hormone replacement therapy. 

References

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  4. Snyder, P. (2020). Causes of secondary hypogonadism in males. UpToDate. Retrieved May 8, 2021, from https://www.uptodate.com/contents/causes-of-secondary-hypogonadism-in-males
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  8. Sizar, O., Schwartz, J. (2020). Hypogonadism. StatPearls. Retrieved June 15, 2021, from https://www.ncbi.nlm.nih.gov/books/NBK532933/

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