eMedicine Specialties > Pediatrics: General Medicine > Endocrinology

Hypogonadism

Stephen Kemp, MD, PhD, Professor, Department of Pediatrics, Section of Pediatric Endocrinology, University of Arkansas and Arkansas Children's Hospital

Updated: Jul 23, 2009

Introduction

Background

Hypogonadism manifests differently in males and in females before and after the onset of puberty. If onset is in prepubertal males and testosterone replacement is not instituted, the individual has features of eunuchoidism, which include sparse body hair, poor development of skeletal muscles, and delay in epiphyseal closure, resulting in long arms and legs. When hypogonadism occurs in postpubertal males, lack of energy and decreased sexual function are the usual concerns. In females with hypogonadism before puberty, failure to progress through puberty or primary amenorrhea is the most common presenting feature. When hypogonadism occurs in postpubertal females, secondary amenorrhea is the usual concern.

Pathophysiology

The gonad (ovary or testis) functions as part of the hypothalamic-pituitary-gonadal axis. A hypothalamic pulse generator resides in the arcuate nucleus, which releases luteinizing hormone (LH)-releasing hormone (LHRH), which is also termed gonadotropin-releasing hormone (GnRH), into the hypothalamic-pituitary portal system. Recent data suggest that a gene named KISS is important in the development of the LHRH secreting cells.¹

In response to these pulses of LHRH, the anterior pituitary secretes follicle-stimulating hormone (FSH) and LH, which, in turn, stimulate gonadal activity. The increase in gonadal hormones results in lowered FSH and LH secretion at the pituitary level, completing the feedback loop. In the testes, LH stimulates Leydig cells to secrete testosterone, whereas FSH is necessary for tubular growth. In the ovaries, LH acts on theca and interstitial cells to produce progestins and androgens, and FSH acts on granulosa cells to stimulate aromatization of these precursor steroids to estrogen.

Hypogonadism may occur if the hypothalamic-pituitary-gonadal axis is interrupted at any level. Hypergonadotropic hypogonadism (primary hypogonadism) results if the gonad does not produce the amount of sex steroid sufficient to suppress secretion of LH and FSH at normal levels. Hypogonadotropic hypogonadism may result from failure of the hypothalamic LHRH pulse generator or from inability of the pituitary to respond with secretion of LH and FSH. Hypogonadotropic hypogonadism is most commonly observed as one aspect of multiple pituitary hormone deficiencies resulting from malformations (eg, septooptic dysplasia, other midline defects) or lesions of the pituitary that are acquired postnatally. In 1944, Kallmann and colleagues first described familial isolated gonadotropin deficiency. Recently, many other genetic causes for hypogonadotropic hypogonadism have been identified.

Frequency

International

In women with hypergonadotropic hypogonadism (ie, gonadal failure), the most common cause of hypogonadism is Turner syndrome, which has an incidence of 1 case per 2,500-10,000 live births. In men with hypergonadotropic hypogonadism, the most common cause is Klinefelter syndrome, which has an incidence of 1 case per 500-1000 live births. Hypogonadotropic hypogonadism is more rare.

Mortality/Morbidity

No increase in mortality is observed in patients with hypogonadism. Morbidity for men and women includes infertility and an increased risk of osteoporosis. In women, an increased risk of severe osteoporosis is noted. In men, hypogonadism causes decreased muscle strength and sexual dysfunction.

Race

No racial predilection has been described.

Sex

Hypergonadotropic hypogonadism is more common in males than in females because the incidence of Klinefelter syndrome (the most common cause of primary hypogonadism in males) is higher than the incidence of Turner syndrome (the most common cause of hypogonadism in females). Incidence of hypogonadotropic hypogonadism is equal in males and females.

Age

Hypogonadism may occur at any age; however, consequences differ according to the age at onset. If hypogonadism occurs prenatally (even if incomplete), sexual ambiguity may result. If hypogonadism occurs before puberty, puberty does not progress. If hypogonadism occurs after puberty, infertility and sexual dysfunction result.

Clinical

History

For both males and females with hypogonadism, determining whether evidence of a genital abnormality is present at birth or determining the timing and extent of puberty is important. In addition, because Kallmann syndrome (hypogonadotropic hypogonadism and anosmia [ie, lack of a sense of smell]) is a common cause of hypogonadotropic hypogonadism, inquiring about the sense of smell is important.

Males
- Specific issues include the presence of developmental anomalies associated with the genital system (eg, hypospadias, micropenis, cryptorchidism). Guidelines for micropenis have been established.²
- For postpubertal males, inquire about the rate of beard growth, libido and sexual function, muscle strength, and energy levels.
- Investigate possible causes of acquired testicular failure (eg, mumps orchitis, trauma, radiation exposure of the head or testes, chemotherapy). Drugs that may interrupt testicular function include agents that interfere with testosterone synthesis, such as spironolactone, cyproterone, marijuana, heroin, and methadone.
Females
- Ask about specific signs associated with Turner syndrome, such as lymphedema, cardiac or renal congenital anomalies, and short growth pattern.
- Determine the age of menarche. Menstrual history is important in postpubertal females.

Physical

Males
- Evaluation of the testes is the most important feature of the physical examination. Determine whether both testes are palpable, their position in the scrotum, and their consistency. Testes size can be quantitated by comparison with testicular models (orchidometer), or their length and width may be measured. Before puberty, testes usually are 1-3 cm³ in volume (approximately 2 cm in length). During puberty, testes grow up to 25 cm³ in size.
- Examining the genitalia for hypospadias is the next important step. Check the scrotum to see if it is completely fused. Finally, evaluate the extent of virilization.
- Puberty should be staged using the Tanner criteria for genitalia, pubic hair, and axillary hair.
- Look for signs of Klinefelter syndrome, such as tall stature (especially if legs are disproportionately long), gynecomastia, small or soft testes, and a eunuchoid body habitus.
Females
- Examination of the genitalia is important.
- Determine the extent of androgenization, which may be adrenal or ovarian in origin and is demonstrated in pubic and axillary hair.
- Determine the extent of estrogenization, as evidenced by breast development and maturation of the vaginal mucosa.
- Look for signs of Turner syndrome, such as short stature, webbing of the neck (eg, pterygium colli), a highly arched palate, short fourth metacarpals, widely spaced nipples, or multiple pigmented nevi.

Causes

Hypogonadotropic hypogonadism

Types of idiopathic hypogonadotropic hypogonadism.
- CNS disorders
- Tumors
  - Craniopharyngioma
  - Germinoma
  - Other germ cell tumors
  - Hypothalamic and optic glioma
  - Astrocytoma
  - Pituitary tumor
- Miscellaneous causes
  - Langerhans histiocytosis
  - Postinfectious lesions of the CNS
  - Vascular abnormalities of the CNS
  - Radiation therapy
  - Congenital malformations (especially associated with craniofacial anomalies)
  - Head trauma
- Genetic causes (see Genetics of hypogonadotropic hypogonadism)
  - Kallmann syndrome (mutation in the KAL [anosmin] gene), with hyposmia or anosmia or without anosmia
  - Congenital adrenal hypoplasia (mutation in the DAX1 gene)
  - Mutations in the PROP1 and HESX1 genes
  - Mutations in the gene coding for the gonadotropin-releasing hormone (GnRH) receptor
  - Isolated luteinizing hormone (LH) deficiency
  - Isolated follicle-stimulating hormone (FSH) deficiency
- Idiopathic and genetic forms of multiple hormone deficiencies
  - Chronic systemic disease and malnutrition
  - Exercise-induced amenorrhea
  - Miscellaneous disorders, including Prader-Willi syndrome, Laurence-Moon syndrome, Bardet-Biedl syndrome,³ functional gonadotropin deficiency (psychogenic amenorrhea, hypothyroidism, diabetes mellitus, Cushing syndrome), hyperprolactinemia, marijuana use, and Gaucher disease
Hypergonadotropic hypogonadism in males
- Klinefelter syndrome
- Inactivating mutations
  - LH beta subunit
  - FSH beta subunit
  - LH receptor
  - FSH receptor
- Other causes of primary testicular failure
  - Chemotherapy
  - Radiation therapy
  - Testicular biosynthetic defects
  - Sertoli-cell-only syndrome
  - LH resistance
  - Anorchism and cryptorchidism
Hypergonadotropic hypogonadism in females
- Turner syndrome
- Inactivating mutations
  - LH beta subunit
  - FSH beta subunit
  - LH receptor
  - FSH receptor
- XX and XY gonadal dysgenesis
  - Familial and sporadic XX gonadal dysgenesis and its variants
  - Familial and sporadic XY gonadal dysgenesis and its variants
- Other causes of primary ovarian failure
  - Premature menopause
  - Radiation therapy
  - Chemotherapy
  - Autoimmune oophoritis
  - Resistant ovary
  - Galactosemia
  - Glycoprotein syndrome type 1
  - FSH-receptor gene mutations
  - LH/human chorionic gonadotropin (hCG) resistance
  - Polycystic ovarian disease
  - Noonan syndrome
Genetics of hypogonadotropic hypogonadism: To date, numerous genes have been identified as causes of hypogonadotropic hypogonadism. The genes include the following:
- KAL is located on the X chromosome, just below the pseudoautosomal region. An abnormality in this gene results in Kallmann syndrome, which is characterized by anosmia and hypogonadotropic hypogonadism.
- The DAX1 gene is associated with X-linked adrenal hypoplasia congenita (hypogonadotropic hypogonadism and adrenal insufficiency).
- GNRHR is the gene associated with the GnRH (LHRH) receptor.
- PC1 is the gene for prohormone convertase 1. Abnormality of this gene causes hypogonadotropic hypogonadism and defects in prohormone processing.
- In addition, mutations in the PROP1 gene have resulted in absence of several pituitary hormones, including growth hormone, thyroid-stimulating hormone, prolactin, and gonadotropins. PROP1 encodes a protein expressed in the embryonic pituitary, which is necessary for function of POU1F1 (formerly PIT1), which codes for a pituitary transcription factor.
- In addition, mutation of the gene HESX1 has been associated with septooptic dysplasia, which may include poor development of the pituitary.

Differential Diagnoses

3-Beta-Hydroxysteroid Dehydrogenase Deficiency	Eating Disorder: Anorexia
5-Alpha-Reductase Deficiency	Growth Failure
Adrenal Hypoplasia	Hypopituitarism
Adrenal Insufficiency	Hypothyroidism
Ambiguous Genitalia and Intersexuality	Klinefelter Syndrome
Amenorrhea	Malnutrition
Androgen Insensitivity Syndrome	Menstruation Disorders
CHARGE Syndrome	Turner Syndrome
Congenital Adrenal Hyperplasia
Denys-Drash Syndrome

Workup

Laboratory Studies

The following studies may be indicated in hypogonadism:

Males
- Determine follicle-stimulating hormone (FSH), luteinizing hormone (LH), prolactin, and testosterone levels and obtain thyroid function test results.
- Examination of seminal fluid, karyotyping, and testicular biopsy may be helpful.
Females
- Determine LH, FSH, prolactin, and estradiol levels and obtain thyroid function test results.
- Karyotyping may be helpful. If gonadotropin levels are elevated, measure antiovarian antibody levels.

Imaging Studies

Pelvic ultrasonography may be helpful in females.

Other Tests

Adrenocorticotropic hormone (ACTH) stimulation testing: In patients in whom a form of congenital adrenal hyperplasia is suspected, adrenal steroid synthesis is best evaluated by performing a cosyntropin (ACTH 1-24) stimulation test. Baseline serum adrenocortical hormone levels are measured, then 0.25 mg of cosyntropin is intravenously injected, and serum hormone levels are remeasured after 60 minutes. Precursor product ratios are compared with those in age-matched control subjects to determine whether a steroidogenic defect is involved in sex hormone synthesis.
Luteinizing-hormone releasing hormone (LHRH) stimulation testing: To distinguish between true hypogonadotropic hypogonadism and constitutional delay in growth and maturation, performing a stimulation test with LHRH may be helpful.
- LHRH is intravenously injected, and LH and FSH levels are determined at 15-minute intervals following LHRH administration.
- A shortened version of the study has been used, in which LHRH is subcutaneously injected, and the specimen for LH and FSH levels is taken at 30-40 minutes.
- Obtaining LHRH for testing over the past several years has been difficult. Some centers have substituted testing LH response to aqueous leuprolide.
Testicular tissue testing: If testes are not palpable and whether any testicular tissue is present is unclear, administering human chorionic gonadotropin (hCG) and measuring testosterone response may be helpful.

Procedures

Bone age may be helpful in distinguishing hypogonadism from constitutional delay in growth and maturation. Timing of onset of puberty is related more to bone age than to chronologic age. Distinguishing hypogonadism from constitutional delay in growth and maturation is often difficult until the bone age is at a point adequate for pubertal development.
Occasionally, testicular biopsy findings are helpful, particularly if azoospermia or oligospermia is present.

Treatment

Medical Care

In prepubertal patients with hypogonadism, treatment is directed at initiating pubertal development at the appropriate age. All such treatment is hormonal replacement therapy. Although the simplest and most successful treatment for both males and females with either hypergonadotropic or hypogonadotrophic hypogonadism is replacement of sex steroids, in hypogonadotropic hypogonadism, the therapy does not confer fertility or, in men, stimulate testicular growth.

An alternative for men with hypogonadotropic hypogonadism has been treatment with pulsatile LHRH or hCG, either of which can stimulate testicular growth. Because such treatment is more complex than testosterone replacement, and because treatment with testosterone does not interfere with later therapy to induce fertility, most male patients with hypogonadotropic hypogonadism prefer to initiate and maintain virilization with testosterone. At a time when fertility is desired, it may be induced with either pulsatile luteinizing hormone-releasing hormone (LHRH) or (more commonly) with a schedule of injections of human chorionic gonadotropin (hCG) and follicle-stimulating hormone (FSH).
In patients with hypergonadotropic hypogonadism, fertility is not possible.

Surgical Care

The only issue of surgical relevance is whether gonadal tissue should be removed.

Because of the significant risk of gonadoblastoma and carcinoma, gonadal tissue should be removed in females with karyotypes containing a Y chromosome. This situation is observed in females with XY gonadal dysgenesis or in patients with Turner syndrome who have a karyotype that contains a Y chromosome (usually in 1 of 2 or more mosaic karyotypes).
Males with nonfunctioning testicular tissue should undergo orchiectomy and replacement with prostheses.

Consultations

Consultation with a reproductive endocrinologist is required for patients who would like to become fertile.
Administration of pulsatile LHRH in adolescents before fertility is desired carries no benefit.

Medication

Treatment of patients with hypergonadotropic hypogonadism involves replacement of sex steroids in both males and females.

For treatment of patients with hypogonadotropic hypogonadism, the usual approach is replacement of sex steroids that initiate development and maintain secondary sex characteristics.

Sex steroid replacement does not result in increased testicular size in males or fertility in either males or females. Gonadotropin or GnRH replacement is offered to the patient when fertility is desired.

Many oral contraceptives can provide estrogen and progesterone in a combination that meets the replacement needs of the patient. Selection of a specific oral contraceptive agent needs to be individualized. All of the contraindications, cautions, and drug interactions for estrogens and progesterones apply, as listed in the tables below.

Testosterone agents

These agents are used for sex steroid replacement in males. All testosterone preparations are regulated as Schedule III controlled substances according to the Anabolic Steroids Control Act.

Testosterone (Andro-LA, Depo-Testosterone)

Several testosterone salts (eg, enanthate, cypionate) are available in a long-acting oil-based preparations. Promotes and maintains secondary sex characteristics in androgen-deficient males.

Dosing

Adult

200-400 mg/mo IM; usually divided q2wk

Pediatric

Initial dose (to initiate puberty): 100 mg/mo IM
Can increase up to adult maintenance dose

Interactions

Glucose metabolism altered with insulin; clotting factor metabolism altered with warfarin

Contraindications

Documented hypersensitivity; severe cardiac or renal disease; benign prostatic hypertrophy with obstruction; males with breast carcinoma; undiagnosed genital bleeding; for use only in males

Precautions

Pregnancy

X - Contraindicated; benefit does not outweigh risk

Precautions

Too frequent or persistent penis erections; impaired liver or renal function

Testosterone patch (Androderm)

Recent addition to the options for testosterone replacement. Several preparations are available, including a scrotal patch (Testoderm) and several patches that may be applied at other sites (Testoderm TTS, Androderm). Patches are changed daily.

Dosing

Adult

Topical patch: 2.5-6 mg/d

Pediatric

Initial dose: 2.5-6 mg/d; dose should start with topical patch that releases 2.5 mg/d in boys who have had minimal prior testosterone exposure; increase gradually to adult dose

Interactions

Glucose metabolism altered with insulin; clotting factor metabolism altered with warfarin

Contraindications

Documented hypersensitivity; breast or prostate carcinoma; severe liver, renal, or cardiac disease; hypercalcemia; for use only in males

Precautions

Pregnancy

X - Contraindicated; benefit does not outweigh risk

Precautions

Too frequent or persistent penis erections; impaired liver or renal function (seen less often with transcutaneous testosterone than with PO or parenteral preparations); monitor serum testosterone levels, growth, and bone age

Testosterone gel (AndroGel)

An androgenic anabolic steroid. The preparation topically is administered as gel.

Dosing

Adult

5-10 g (delivers approximately 50-100 mg) applied topically every am to shoulders, upper arms, and abdomen

Pediatric

Not established

Interactions

None reported

Contraindications

Documented hypersensitivity; breast or prostate carcinoma; female patients; boys <18 y; just after application, avoid direct contact with women who are pregnant

Precautions

Pregnancy

X - Contraindicated; benefit does not outweigh risk

Precautions

Impaired liver function (seen less often with transcutaneous testosterone compared to oral or parenteral preparations); congestive heart failure; elderly patients; benign prostatic hypertrophy

Estrogen agents

These agents are used for sex steroid replacement in females.

Ethinyl estradiol (Estrace)

May initiate puberty in girls. A small unopposed dose (0.02 mg) is administered daily for 3-6 mo, then the dose is increased and cycled. After the first 6 mo, adding progestogen is often helpful.

Dosing

Adult

0.02-0.1 mg/d PO alone for first 14-20 d of theoretical menstrual cycle, followed by 12-14 d of additional progesterone preparation

Pediatric

0.02-0.1 mg/d PO
May be administered unopposed initially; after 6-12 mo, should be administered alone for first 14-20 d of cycle, followed by addition of 12-14 d of progesterone

Interactions

May reduce hypoprothrombinemic effects of anticoagulants; estrogen levels may be reduced with coadministration of barbiturates, rifampin, and other agents that induce hepatic microsomal enzymes; an increase in corticosteroid levels may occur when administered concurrently with ethinyl estradiol; use of ethinyl estradiol with hydantoins may cause spotting, breakthrough bleeding, and pregnancy; increase in fluid retention caused by estrogen intake may reduce seizure control

Contraindications

Documented hypersensitivity; known or suspected pregnancy; breast cancer; undiagnosed abnormal genital bleeding; active thrombophlebitis or thromboembolic disorders; history of thrombophlebitis, thrombosis, or thromboembolic disorders associated with previous estrogen use (except when used in treatment of breast or prostatic malignancy); for females only; smoking cigarettes

Precautions

Pregnancy

X - Contraindicated; benefit does not outweigh risk

Precautions

Caution in hepatic impairment, migraine, seizure disorders, cerebrovascular disorders, breast cancer, or thromboembolic disease

Estradiol, transdermal (Climara, Vivelle, Esclim, Alora, FemPatch)

May initiate puberty in girls. Initially, a 0.05-mg patch may be applied 1-2 times/wk. After 6-12 mo, dose may be increased and cycled. After first 6 mo, adding progestogen is often helpful. A very low starting dose of estrogen is desired in young girls with bone ages at or below 12-13 y. Starting at higher doses may cause rapid epiphyseal maturation. If necessary, patches with a matrix-release mechanism (eg, Climara, Vivelle) may be cut to deliver a smaller dose. In the case of the Vivelle dot, half of the dot may be covered in order to lower the amount of estrogen absorbed.

Dosing

Adult

Apply topical patch that delivers estradiol at rate of 0.025-0.1 mg/d; replace qwk or 2 times/wk according to specific patch directions

Pediatric

Administer as in adults; start with lowest dose

Interactions

May reduce hypoprothrombinemic effect of anticoagulants; coadministration of barbiturates, rifampin, and other agents that induce hepatic microsomal enzymes may reduce estrogen levels; pharmacologic and toxicologic effects of corticosteroids may occur because of estrogen-induced inactivation of hepatic P-450 enzyme; loss of seizure control has been noted when administered concurrently with hydantoins

Contraindications

Precautions

Pregnancy

X - Contraindicated; benefit does not outweigh risk

Precautions

Certain patients may develop undesirable manifestations of excessive estrogenic stimulation, such as abnormal or excessive uterine bleeding or mastodynia; estrogens may cause some degree of fluid retention (exercise caution); prolonged unopposed estrogen therapy may increase risk of endometrial hyperplasia

Conjugated estrogen (Premarin)

May initiate puberty in girls. A small unopposed dose is administered for 3-6 mo, then the dose is increased. After the first 6 mo, adding progestogen is often helpful.

Dosing

Adult

0.625-1.25 mg/d PO

Pediatric

May be administered PO to initiate pubertal development in girls starting at lower dose (0.3 mg/d) and advancing to higher dose (0.625 mg/d)
When dose is increased, may add progesterone
After 1-2 y, may increase to adult dose (1.25 mg/d on days 1-21 of cycle)

Interactions

Contraindications

Precautions

Pregnancy

X - Contraindicated; benefit does not outweigh risk

Precautions

Progesterone agents

These agents are added during the last 12-14 days of the menstrual cycle.

Norethindrone (Aygestin)

Transforms proliferative into secretory endometrium.

Dosing

Adult

5 mg/d PO during final 12-14 d of menstrual cycle

Pediatric

Administer as in adults

Interactions

Hepatic metabolism induced with aminoglutethimide or rifampin

Contraindications

Documented hypersensitivity; thromboembolic disorders; pregnancy; missed abortion; breast cancer; undiagnosed vaginal bleeding

Precautions

Pregnancy

X - Contraindicated; benefit does not outweigh risk

Precautions

During lactation, impaired liver function, congestive heart failure, or hypertension; fluid retention, depression, glucose intolerance, and thromboembolic phenomena may occur; perform follow-up testing with Papanicolaou smears

Medroxyprogesterone (Provera, Amen, Cycrin)

Transforms proliferative into secretory endometrium.

Dosing

Adult

5-10 mg/d PO during last 12-14 d of menstrual cycle

Pediatric

Administer as in adults

Interactions

Hepatic metabolism induced with aminoglutethimide or rifampin

Contraindications

Documented hypersensitivity; cerebral apoplexy; undiagnosed vaginal bleeding; thrombophlebitis; liver dysfunction

Precautions

Pregnancy

X - Contraindicated; benefit does not outweigh risk

Precautions

Follow-up

Further Outpatient Care

Reevaluate patients with hypogonadism receiving hormone replacement therapy every 6-12 months.

Complications

In men, complications of untreated hypogonadism include loss of libido, failure to achieve physical strength, the social implications of failing to go through puberty with peers (if hypogonadism occurs before puberty), and osteoporosis. In addition, if hypogonadism occurs before epiphyseal closure, the result is usually tall stature with a eunuchoid body habitus. Even treated males with primary hypogonadism are infertile. However, men who have hypogonadism due to hypothalamic or pituitary dysfunction can potentially become fertile with administration of gonadotropins.
In women with hypogonadism, complications include the social implication of failing to go through puberty with peers (if hypogonadism occurs before puberty). An additional concern for untreated women is osteoporosis, which can be avoided with estrogen replacement. Women who have hypogonadism because of hypothalamic or pituitary dysfunction can potentially become fertile with administration of gonadotropins. Women with primary hypogonadism are infertile; however, with in vitro fertilization using a donor ovum, these women can carry an infant to term.
Osteoporosis has an earlier onset in individuals with hypogonadism; hence, bone mineral density should be compared with age-matched normative standards, and followed longitudinally. Prescribe treatment using appropriate therapeutic interventions.

Prognosis

Men and women with hypogonadism can lead a normal life with hormone replacement.
Approximately 20-25% of females with Turner syndrome have some spontaneous puberty. Spontaneous estrogenization occurs more commonly in women with mosaic karyotypes and those karyotypes with an abnormal second X chromosome, such as 46,XXiq or 46,XXip. Reports exist of women with mosaic Turner syndrome becoming pregnant without in vitro fertilization.

Patient Education

For excellent patient education resources, visit eMedicine's Men's Health Center and Women's Health Center. Also, see eMedicine's patient education articles Impotence/Erectile Dysfunction and Amenorrhea.

Miscellaneous

Special Concerns

Both males and females with hypogonadotropic hypogonadism (hypogonadism at the pituitary or hypothalamic level) can potentially become fertile. Induction of fertility requires involvement of a specialist in reproductive endocrinology.

Multimedia

Media file 1: Types of idiopathic hypogonadotropic hypogonadism.

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Keywords

hypogonadism, ovarian failure, testicular failure, gonadal failure, eunuchoidism, primary amenorrhea, secondary amenorrhea, decreased sexual function, sparse body hair, delayed epiphyseal closure, hypergonadotropic hypogonadism, hypogonadotropic hypogonadism, gonadotropin deficiency, Turner syndrome, Turner's syndrome, Klinefelter syndrome, Klinefelter's syndrome, infertility, sexual dysfunction, decreased muscular strength, primary hypogonadism, sexual ambiguity, Kallmann syndrome, Kallmann's syndrome, genital abnormality, hypospadias, cryptorchidism, micropenis, treatment, diagnosis

Contributor Information and Disclosures

Author

Stephen Kemp, MD, PhD, Professor, Department of Pediatrics, Section of Pediatric Endocrinology, University of Arkansas and Arkansas Children's Hospital
Stephen Kemp, MD, PhD is a member of the following medical societies: American Academy of Pediatrics, American Association of Clinical Endocrinologists, American Pediatric Society, Endocrine Society, Phi Beta Kappa, Southern Medical Association, and Southern Society for Pediatric Research
Disclosure: Genentech, Inc. Honoraria Speaking and teaching; Pfizer, Inc. Honoraria Consulting

Medical Editor

Phyllis W Speiser, MD, Chief of Pediatric Endocrinology, Schneider Children's Hospital; Professor of Pediatrics, New York University School of Medicine
Phyllis W Speiser, MD is a member of the following medical societies: American Association of Clinical Endocrinologists, Endocrine Society, Lawson-Wilkins Pediatric Endocrine Society, and Society for Pediatric Research
Disclosure: Nothing to disclose.

Pharmacy Editor

Mary L Windle, PharmD, Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy, Pharmacy Editor, eMedicine
Disclosure: Pfizer Inc Stock Investment from financial planner; Avanir Pharma Stock Investment from financial planner ; WebMD Salary and stock Employment and investment from financial planner

Managing Editor

Barry B Bercu, MD, Professor, Departments of Pediatrics, Molecular Pharmacology and Physiology, University of South Florida College of Medicine, All Children's Hospital
Barry B Bercu, MD is a member of the following medical societies: American Academy of Pediatrics, American Association of Clinical Endocrinologists, American Federation for Clinical Research, American Medical Association, American Pediatric Society, Association of Clinical Scientists, Endocrine Society, Florida Medical Association, Lawson-Wilkins Pediatric Endocrine Society, Pituitary Society, Society for Pediatric Research, Society for the Study of Reproduction, and Southern Society for Pediatric Research
Disclosure: Nothing to disclose.

CME Editor

Merrily P M Poth, MD, Professor, Department of Pediatrics and Neuroscience, Uniformed Services University of the Health Sciences
Merrily P M Poth, MD is a member of the following medical societies: American Academy of Pediatrics, Endocrine Society, and Lawson-Wilkins Pediatric Endocrine Society
Disclosure: Nothing to disclose.

Chief Editor

Bruce Buehler, MD, Professor, Department of Pediatrics, Pathology and Microbiology, Executive Director, Hattie B Munroe Center for Human Genetics, University of Nebraska Medical Center
Bruce Buehler, MD is a member of the following medical societies: American Academy for Cerebral Palsy and Developmental Medicine, American Academy of Pediatrics, American Association on Mental Retardation, American College of Medical Genetics, American College of Physician Executives, American Medical Association, and Nebraska Medical Association
Disclosure: Nothing to disclose.

Further Reading