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Primary amenorrhea hypogonadotropic hypogonadism – Amenorrhea: Evaluation and Treatment

Primary versus secondary amenorrhea. Prolactin may be low in cases of pituitary destruction or elevated if a space-filling lesion compresses the pituitary stalk and blocks dopaminergic inhibition of prolactin.

Lucas Cox
Sunday, October 21, 2018
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  • Depending on primary amenorrhea hypogonadotropic hypogonadism etiology of the hypothalamic dysfunction, these women are typically normal height, but some if not treated may develop short stature. It is important to note that there are few studies about the use of gonadotropins in adolescents, and most them are small case series of boys with HH who received pubertal induction with gonadotropins at various times, and thus further studies are needed.

  • False-negative results may also be caused by the variant effect. Causes of primary amenorrhea should be evaluated in the context of the presence or absence of secondary sexual characteristics.

  • Clinical presentation of HH depends on the time of onset ie, congenital vs acquiredthe severity of the defect, and the presence of associated conditions. Prim Care.

  • Secondary amenorrhea is the absence of menses for three months in women with previously normal menstruation and for nine months in women with previous oligomenorrhea. Many pituitary hormones, most notably growth hormone GH and adrenocorticotropic hormone ACTHvary throughout the day and are best assessed using dynamic testing to either stimulate or suppress hormonal response.

Evaluation

Gynecol Endocrinol. Hyperprolactinemia indicates a prolactinoma or other pituitary adenoma. Rudimentary or absent uterus; pubic hair.

Adequate calcium and vitamin D intake are recommended for these patients. First morning specimens are recommended. Algorithm for the evaluation of secondary amenorrhea. Initial growth acceleration 8 to N Engl J Med. Table 4 3615 includes the differential diagnosis of primary amenorrhea.

  • Endocr Rev. Treatment of microadenomas should focus on management of infertility, galactorrhea, and breast discomfort.

  • Histamine H 2 receptor blockers.

  • Medications usually raise prolactin levels to less than ng per mL.

  • Read the Issue. Prolactin is mildly elevated by stress, herpes simplex virus HSV infections in the chest wall, and numerous drugs, including dopamine agonists, proton pump inhibitors, antipsychotics risperidone, phenothiazines, haloperidolantihypertensives methyldopa, reserpine, verapamilestrogens, and illicit drugs amphetamines, cannabinoids, opiates, etc.

  • Intramuscular injections of long-acting testosterone esters testosterone cypionate or enanthate are commonly used.

  • Illustrations by Renee Cannon.

Hypogonzdotropic diagnosis of hypogonadotropic hypogonadism is fairly simple, elucidation of the cause of disease in patients is challenging. First morning specimens are recommended. Nonclassic congenital adrenal hyperplasia. False-positive and false-negative results for urine pregnancy tests can occur and may result in a misdiagnosis. In particular, does your patient take any medications — OTC drugs or Herbals — that might affect the lab results? Gonadotropin levels can further help determine the source of the abnormality.

Traggiai C, Stanhope R. Apr 15, Issue. Some space-filling lesions compress the pituitary stalk and prevent negative feedback from dopamine, leading to hyperprolactinemia. Cyclic abdominal pain; breast changes. Opiates, cocaine. In adolescent athletes, the bone loss occurs during peak bone mass development and may not be reversible.

Case Report

Family history. Other causes of outflow tract obstruction include cervical stenosis and obstructive fibroids or polyps. Algorithm for the evaluation of secondary amenorrhea.

LH and FSH should be measured early in the follicular phase of the cycle, if ammenorrhea. Infiltrative diseases: hemochromatosis, sarcoidosis, granulomatous diseases, histiocytosis X, lymphocytic hypophysitis. Postmenopausal ovarian failure. Digenic mutations account for variable phenotypes in idiopathic hypogonadotropic hypogonadism. The possibility of nutritional disorders or an undiagnosed chronic illness that may affect the hypothalamic GnRH pulse generator should be evaluated in patients with HH. Impaired fertility caused by endocrine dysfunction in women. Exogenous androgens.

Traggiai C, Stanhope R. A person viewing it online may make one printout of the material and may use that printout hypogpnadotropic for his or her personal, non-commercial reference. A reduced fT4 confirms hypothyroidism. Any findings suggestive of pituitary dysfunction should prompt an MRI to look for a pituitary lesion. Register now at no charge to access unlimited clinical news, full-length features, case studies, conference coverage, and more. However, the effects of subclinical hypothyroidism on menstruation and fertility are unclear, and abnormal thyroid hormone levels can affect prolactin levels; therefore, physicians should consider measuring thyroid-stimulating hormone TSH levels.

Differential Diagnosis of Primary Amenorrhea

Menarche and menstrual history mother and sisters. Patient information: See related handout on amenorrheawritten by the authors of this article. Low gonadotropin and estradiol levels resulting in primary amenorrhea and poor pubertal development suggested the diagnosis of a severe form of HH in this young lady.

Young women with HH are at risk for bone hypogonadism and fracture. Full replacement dose of estrogen and progesterone is attained with 0. Menstrual hy;ogonadism after metformin therapy in polycystic ovary syndrome. Work-up of a patient with primary amenorrhea due to hypothalamic causes is diagnosis of exclusion. Secondary amenorrhea is the absence of menses for three months in women with previously normal menstruation and for nine months in women with previous oligomenorrhea.

This amenorrrhea occurs when high concentrations of hCG isoforms in urine hCG beta core fragment are not recognized by both antibodies in the assay. An elevated prolactin result should prompt a physician to perform an MRI in search of a pituitary adenoma. Breast stimulation. Various causes of disease are identified by patient history.

It furthers primary amenorrhea hypogonadotropic hypogonadism University's objective of excellence in research, scholarship, and education by publishing worldwide. Advanced Search. Pubic hair and breast development were Tanner stage II. Women with polycystic ovary syndrome should be tested for glucose intolerance. It is important to differentiate adult-onset HH, characterized by frankly low serum testosterone levels in the presence of low or normal gonadotropins, from the progressive testosterone deficiency observed in a small minority of aging men, known as late-onset hypogonadism. Although primarily a disease of females, eating disorders such as anorexia nervosa are increasingly being recognized in males and are associated with hypogonadism.

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Prevalence hypogonadotropic hypogonadism associated disease risks. If patient is not pregnant, amenorrnea patient should undergo imaging studies to detect the presence and absence of uterus and gonads. Any disruption in this interaction can cause amenorrhea. Moreover, the importance of low levels of leptin, a hormone secreted by adipocytes that regulates energy homeostasis, in the pathophysiology of hypothalamic amenorrhea was clearly demonstrated by evidence of a significant improvement of the reproductive and neuroendocrine functions in women with hypothalamic amenorrhea after exogenous recombinant leptin replacement 21 Anosmia can be easily diagnosed by questioning the patient, whereas olfactometry, such as University of Pennsylvania Smell Identification Test, is necessary to determine reliably whether olfaction is normal or partially defective.

Congenital idiopathic hypogonadotropic hypogonadism: evidence of defects in the hypothalamus, pituitary, and testes. Another option for patients with partial pubertal development is to start with hCG alone for 6 months and subsequently add FSH if azoospermia persists. Navigate this Article. Earn up to 6 CME credits per issue. In contrast, patients with congenital IHH have normal linear growth during childhood, and despite the absence of the pubertal growth spurt, short stature is not a common finding. Arch Dis Child.

  • Genetics basis for GnRH-dependent pubertal disorders in humans. Prolactin levels should be checked in most patients.

  • Patients with hypothalamic primary amenorrhea have a normal prepubescent uterus, but, prior to further work-up for hypothalamic primary amenorrhea, patients with a detectable uterus should be evaluated for hypothyroidism and prolactinemia.

  • After pregnancy, thyroid disease, and hyperprolactinemia are eliminated as potential diagnoses, the remaining causes of secondary amenorrhea are classified as normogonadotropic amenorrhea, hypogonadotropic hypogonadism, and hypergonadotropic hypogonadism; each is associated with specific etiologies Table 4 36 ,

  • A blood-based polyamine signature associated with MEN1 duodenopancreatic neuroendocrine tumor progression.

Androgen-secreting tumor. Elevations of free alpha-subunit may also occur within 24 hours of ovulation, in end stage renal disease, in hypothyroidism due to elevated TSH, and in women undergoing assisted reproduction or IVF. Amenorrhea is the absence of menstrual blood flow. Constitutional delay of growth and puberty commonly causes primary amenorrhea in patients with no sexual development. The treatment of primary and secondary amenorrhea is based on the causative factor. Amenorrhea: Evaluation and Treatment.

Prolactin levels should be checked in most patients. Microadenomas are slow growing and rarely malignant. Progestogen challenge test. Progesterone micronized.

Background

Young athletes may develop a combination of hypogonadofropic conditions called the female athlete triad that includes an eating disorder, amenorrhea, and osteoporosis. If asymptomatic microadenomas smaller than 10 mm are found on MRI, repeat prolactin measurements and imaging should be performed to monitor for progression. Heiman completed a family practice residency at the University of Virginia School of Medicine, Charlottesville. Identification of mutations in several other genes by DNA sequencing analysis helps confirm other congenital GnRH deficiencies.

Once pituitary causes are excluded, correlation of primady testing with patient history e. Simpson J, Rajkovic A. Show More. Adequate calcium and vitamin D intake are recommended for these patients. Delayed sexual development: a study of patients. Am Fam Physician. The primary etiology of PCOS is unknown, but resistance to insulin is thought to be a fundamental component.

Bone mineral density primarj the lumbar spine, femoral neck, and hip is recommended at the initial diagnosis of Primary amenorrhea hypogonadotropic hypogonadism and after 1 to 2 years of sex steroid therapy in hypogonadal patients with osteoporosis or low trauma fracture Select Format Select format. Congenital hypogonadism may be particularly detrimental to the skeleton because it may lead to failure to achieve peak bone mass, in addition to loss of established bone mass. Algorithm for the evaluation of primary amenorrhea. Get immediate access, anytime, anywhere.

  • A more recent article on amenorrhea is available.

  • At a Glance Amenorrhea is the absence of menstrual blood flow.

  • Philadelphia, Pa. Read the Issue.

  • Prolactin secreting pituitary adenomas can induce hyperprolactinemia, which suppresses LH and FSH, leading to amenorrhea. A family history significant for delayed puberty should prompt a genetic work-up.

Endocrinol Metab Clin North Am. At a Glance Amenorrhea is the absence of menstrual blood flow. A withdrawal bleed usually occurs two to seven days after the challenge test. Elevations of free alpha-subunit may also occur within 24 hours of ovulation, in end stage renal disease, in hypothyroidism due to elevated TSH, and in women undergoing assisted reproduction or IVF. Other clinical signs of thyroid disease are usually noted before amenorrhea presents. The diagnosis of PCOS is primarily clinical, although laboratory studies may be needed to rule out other causes of hyperandrogenism Table 5 6 ,

In patients with primary amenorrhea, the presence or absence of sexual development should direct the evaluation. Pubertal history e. Various causes of disease are identified by assessing other pituitary hormones. In: Clinical gynecologic endocrinology and infertility. Progestogen challenge test. Table 1.

Evaluation

Idiopathic gonadotrophin deficiency: genetic questions addressed through phenotypic characterization. Role of gonadotropin-releasing hormone and human chorionic gonadotropin stimulation tests in differentiating patients with hypogonadotropic hypogonadism from those with constitutional delay of growth and puberty. Premature ovarian failure.

Progesterone is given orally for up to 1 week. Delayed puberty: analysis of a large case series from an academic center. Pubertal history e. Obstet Gynecol Surv. Prolactin inhibits gonadotropin function, thus, causing amenorrhea in nursing mothers and patients with prolactinomas. Evaluation of Primary Amenorrhea Figure 1.

Chronic illnesses, such as anemia, hypertension, and liver and kidney diseases, affect estrogen concentrations. Mild hypothyroidism is more often associated with hypermenorrhea or oligomenorrhea than with amenorrhea. False-negative results may also be caused by the variant effect. Delayed puberty.

Differential Diagnosis of Primary Amenorrhea

Functional hypothalamic amenorrhea is a frequent cause of acquired female infertility, typically manifested as amenorrhea of 6-month duration amsnorrhea longer, low or normal gonadotropin levels, and hypoestrogenemia primary amenorrhea hypogonadotropic hypogonadism organic abnormalities 19 Although widely used, the practical value of the GnRH test has been questionable because of its low cost-effectiveness. In particular, in response to GnRH therapy, women with congenital hypothalamic amenorrhea showed significantly increased free alpha subunit compared to women with constitutional delay or acquired hypothalamic amenorrhea. In these women, GnRH secretion is disrupted, leading to decreased gonadotropin and sex steroid secretion.

  • The reversible form of HH should be suspected if testicular volume increases during testosterone administration or in the absence of endocrine therapy.

  • Log in Best Value! The primary etiology of PCOS is unknown, but resistance to insulin is thought to be a fundamental component.

  • Two common causes of normogonadotropic amenorrhea are outflow tract obstruction and hyperandrogenic chronic anovulation.

  • If a patient has secondary amenorrhea, pregnancy should be ruled out.

  • Exercise, weight loss, current or previous chronic illness, illicit drug use. Psychosocial stressors; nutritional and exercise history.

Hyperandrogenic anovulation. Hypogonadotropic hypogonadism. Premature ovarian failure: an update. Delayed puberty. Bone mineral changes in young women with hypothalamic amenorrhea treated with oral contraceptives, medroxyprogesterone, or placebo over 12 months.

Patients with hypothalamic disease will respond to GnRH primary amenorrhea hypogonadotropic hypogonadism by secreting LH and FSH, whereas patients with pituitary disease will not secrete gonadotropins in response to releasing hormone. A female patient with primary amenorrhea and sexual development, including pubic hair, should be evaluated for the presence of a uterus and vagina. Digenic mutations account for variable phenotypes in idiopathic hypogonadotropic hypogonadism. Other baseline measurements anti-Mullerian hormone, testosterone, FSH, and LH were not useful for such discrimination. The maintenance dose for adult males is — mg im every 2—3 weeks or mg of testosterone undecanoate every 3 months. However, the effects of subclinical hypothyroidism on menstruation and fertility are unclear, and abnormal thyroid hormone levels can affect prolactin levels; therefore, physicians should consider measuring thyroid-stimulating hormone TSH levels. Because functional hypothalamic primary amenorrhea is usually a transient disease, conception is possible.

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If a patient has secondary amenorrhea, pregnancy should be ruled out. Exogenous androgens. FSH and LH responses were similar in both sets of women. Causes of primary amenorrhea should be evaluated in the context of the presence or absence of secondary sexual characteristics. Constitutional delay of growth and puberty.

Google Scholar. No genetic defect in genes associated primary amenorrhea hypogonadotropic hypogonadism congenital IHH has been identified in this group of patients N Engl J Med. Mol Cell Endocrinol. HH may result from either absent or inadequate hypothalamic GnRH secretion or failure of pituitary gonadotropin secretion. In: Clinical gynecologic endocrinology and infertility.

This content is owned by the AAFP. Low concentrations of FSH, LH, and estradiol or a failed progesterone challenge test are suggestive amenorrhea hypothalamic amenorrhea. Elevation of papilla only; no pubic hair. Patients younger than 30 years should receive a karyotype analysis to rule out the presence of a Y chromosome and the need for removal of gonadal tissue. Instead, they interfere with one antibody and cause a false-negative result. A thorough history and physical examination as well as laboratory testing can help narrow the differential diagnosis of amenorrhea. Similar to patients with eating disorders, athletes with continued amenorrhea are at risk of bone loss.

GnRH stimulation testing may help differentiate between pituitary and hypogonadism causes of HH. Evaluation of Primary Amenorrhea Figure 1. Hypergonadotropic hypogonadism elevated FSH and LH levels in patients with primary amenorrhea is caused by gonadal dysgenesis or premature ovarian failure. Gymnasts exhibit higher bone mass than runners despite similar prevalence of amenorrhea and oligomenorrhea. Two common causes of normogonadotropic amenorrhea are outflow tract obstruction and hyperandrogenic chronic anovulation. Issue Section:. Women with premature ovarian failure have an increased risk of osteoporosis and heart disease.

Patients who have not developed yhpogonadotropic sex characteristics, especially the absence of breast development, and have not established periodic menstruation primary amenorrhea hypogonadotropic hypogonadism 13 years of age should also be worked up for primary amenorrhea. Pubertal changes typically occur over a three-year period and can be measured using Tanner staging. Treatment of hypothyroidism should restore menses, but this may take several months.

Diagnosis of polycystic ovarian syndrome. Other laboratory testing should be determined based on the individual patient. Chronic liver disease. If the patient has normal pubertal development and a uterus, the most common etiology is congenital outflow tract obstruction with a transverse vaginal septum or imperforate hymen.

  • Impaired endothelial function in young women with premature ovarian failure: normalization with hormone therapy. Young women with HH are at risk for bone loss and fracture.

  • Family history. N Engl J Med.

  • No genetic defect in genes associated with congenital IHH has been identified in this group of patients Endocr Rev.

  • If testes are present, they should be removed because of the high risk of malignant transformation after puberty. Chronic illnesses, such as anemia, hypertension, and liver and kidney diseases, affect estrogen concentrations.

  • Gonadotropin-releasing hormone deficiency in the human idiopathic hypogonadotropic hypogonadism and Kallmann's syndrome : pathophysiological and genetic considerations. The Endocrine Society has achieved Accreditation with Commendation.

Sign In or Create hypoonadism Account. Central anatomic defects and systemic diseases were excluded by routine tests and a normal brain imaging. Notably, the intra-subcutaneous route of administration is as effective as im. Most commonly, however, the diagnosis cannot be confirmed until the expected time of puberty onset, except in the neonatal period, when gonadotropin and sexual steroid levels are expected to be elevated. Women with secondary amenorrhea should receive pregnancy tests. Polycystic ovary syndrome. A brief discontinuation of hormonal therapy to assess reversibility is rational in patients with HH.

Women with excessive weight loss aamenorrhea be screened for eating disorders and treated if anorexia nervosa or bulimia nervosa is diagnosed. Purchase Access: See My Options close. Patients with primary amenorrhea due to hypothalamic abnormalities often have delayed development of secondary sex characteristics. Because functional hypothalamic primary amenorrhea is usually a transient disease, conception is possible. Premature ovarian failure is characterized by amenorrhea, hypoestrogenism, and increased gonadotropin levels occurring before 40 years of age and is not always irreversible 27 0.

Young athletes may develop a combination of health conditions called the female athlete triad that includes an eating disorder, amenorrhea, and osteoporosis. Mayo Clin Proc. Exercise, weight loss, current or previous chronic illness, illicit drug use. False-negative results may also be caused by the variant effect.

Khan academy macrocytic anemia hypothyroidism low LH and FSH results may occur in patients taking oral contraceptives and hormone treatments. Secondary amenorrhea is the absence of menses for three months in women with previously normal menstruation and for nine months in women with previous oligomenorrhea. A reduced fT4 confirms hypothyroidism. Liver failure. Does age at natural menopause affect mortality from ischemic heart disease?. Next: Arrhythmogenic Right Ventricular Dyplasia. Arch Dis Child.

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Amenorhea with hypothalamic primary amenorrhea have a normal prepubescent uterus, but, primary amenorrhea hypogonadotropic hypogonadism to further work-up for hypothalamic primary amenorrhea, patients with a detectable uterus should be evaluated for hypothyroidism and prolactinemia. Congenital IHH is a clinically and genetically heterogeneous disorder. There is no confirmatory test for most causes of functional hypothalamic amenorrhea. Early induction of spermatogenesis may reduce the time required for appearance of sperm and the need for prolonged cycles of gonadotropin treatment in adult life. Evaluate for androgen-secreting tumor. Hyperinsulinemia in polycystic ovary syndrome correlates with increased cardiovascular risk independent of obesity.

Androgen-secreting tumor. Breast stimulation. Multiple, depending on medication. Tumors: prolactinomas, Rathke's pouch cysts, craniopharyngiomas, germinomas, teratomas, meningiomas, gliomas, astrocytomas, metastatic tumors breast, lung, prostate. Algorithm for the evaluation of primary amenorrhea.

  • Other rare causes of pure gonadal dysgenesis can occur with a 46,XY or XX karyotype.

  • Purchase Access: See My Options close.

  • Close mobile search navigation Article Navigation. Hypergonadotropic hypogonadism.

  • Google Scholar Crossref. Fertil Steril.

  • Inflammatory bowel disease.

A karyotype analysis hypogonavotropic needed to determine proper treatment. Inflammatory bowel disease. The idiopathic form of adult-onset Primary amenorrhea hypogonadotropic hypogonadism is a rare disorder characterized by an isolated failure of gonadotropin secretion occurring after an otherwise normal sexual maturation in men in whom anatomical, systemic, or functional causes had been ruled out Amenorrhea is the absence of menstrual blood flow. Oxford University Press is a department of the University of Oxford. Next: Arrhythmogenic Right Ventricular Dyplasia.

Hypogonadotropic hypogonadism primafy management of pituitary tumors: recent advances. Table 1. Decreased leptin levels in normal weight women with hypothalamic amenorrhea: the effects of body composition and nutritional intake. Patients who have not developed secondary sex characteristics, especially the absence of breast development, and have not established periodic menstruation by 13 years of age should also be worked up for primary amenorrhea. Log in. Bone mineral changes in young women with hypothalamic amenorrhea treated with oral contraceptives, medroxyprogesterone, or placebo over 12 months.

Metformin therapy decreases hyperandrogenism and hyperinsulinemia in women with polycystic ovary syndrome. Hypogonxdism 2 1 — 36 is an algorithm for the evaluation of secondary amenorrhea. Androgen insensitivity syndrome. The risk of amenorrhea is lower with subclinical hypothyroidism than with overt disease. LH and FSH are episodically released from the pituitary, and concentrations may vary, depending on when they are measured during the day.

Significantly elevated testosterone or dehydroepiandrosterone sulfate levels indicate hypogonadotropic hypogonadism possible androgen-secreting tumor ovarian or adrenal. The primary treatment for PCOS is weight loss through diet and exercise. It may take months to see these results, however. Treatment goals include prevention of complications such as osteoporosis, endometrial hyperplasia, and heart disease; preservation of fertility; and, in primary amenorrhea, progression of normal pubertal development. Polycystic ovary syndrome PCOS is the most common cause of hyperandrogenic chronic anovulation.

Hypergonadotropic hypogonadism. Galactorrhea; headache and visual disturbances. If a patient has secondary amenorrhea, pregnancy should be ruled out. No genetic defect in genes associated with congenital IHH has been identified in this group of patients Already a member or subscriber? Androgen insensitivity syndrome. Delayed puberty.

Simpson J, Rajkovic A. Prescription drug use. Delayed puberty.

There is some variability in the cutoff values that can affect sensitivity and specificity of the test. Menstrual cyclicity after metformin therapy in polycystic ovary amenorryea. Central anatomic defects and systemic diseases were excluded by routine tests and a normal brain imaging. Permissions Icon Permissions. If the patient has a normal uterus, outflow tract obstruction should be considered. Role of gonadotropin-releasing hormone and human chorionic gonadotropin stimulation tests in differentiating patients with hypogonadotropic hypogonadism from those with constitutional delay of growth and puberty.

Rudimentary or absent uterus; pubic hair. Polycystic ovary syndrome. Bronchogenic e. Want to view more content from Cancer Therapy Advisor? Instead, they interfere with one antibody and cause a false-negative result. The authors thank Barbara S. The treatment of primary and secondary amenorrhea is based on the causative factor.

Routine endocrine screening for patients with karyotypically normal hypotonadotropic premature ovarian failure. Treatment of microadenomas should focus on management of infertility, galactorrhea, and breast discomfort. In patients khan academy macrocytic anemia hypothyroidism whom fertility is desired, induction of gonadotropin secretion by pulsatile GnRH or treatment with exogenous gonadotropin is the current hormonal treatment of choice. Log in Best Value! Full replacement dose of estrogen and progesterone is attained with 0. Two novel missense mutations in g protein-coupled receptor 54 in a patient with hypogonadotropic hypogonadism.

The hypogonadotropic hypogonadism treatment for PCOS is weight loss through diet and exercise. Chronic liver disease. TABLE 2 History and Physical Examination Findings Associated with Amenorrhea Findings Associations Patient history Exercise, weight loss, current or previous chronic illness, illicit drug use Hypothalamic amenorrhea Menarche and menstrual history Primary versus secondary amenorrhea Prescription drug use Multiple, depending on medication Previous central nervous system chemotherapy or radiation Hypothalamic amenorrhea Previous pelvic radiation Premature ovarian failure Psychosocial stressors; nutritional and exercise history Anorexia or bulimia nervosa Sexual activity Pregnancy Family history Genetic defects Multiple causes of primary amenorrhea Pubic hair pattern Androgen insensitivity syndrome Infertility Multiple Menarche and menstrual history mother and sisters Constitutional delay of growth and puberty Pubertal history e. Artificially low LH and FSH results may occur in patients taking oral contraceptives and hormone treatments.

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  • LH and FSH should be measured early in the follicular phase of the cycle, if possible. Androgen insensitivity syndrome.

  • J Clin Epidemiol.

  • More specifically, gonadotrope development may be impaired by mutations in Hesx1 and Prop-1, producing isolated gonadotropin deficiency.

Hypogondism with a detectable uterus should be evaluated for hypothyroidism and prolactinemia. Bone mineral changes in young women with hypothalamic amenorrhea treated with oral contraceptives, medroxyprogesterone, or placebo over 12 months. C 1 — 36 Women with polycystic ovary syndrome should be tested for glucose intolerance. False-positive and false-negative results in urine pregnancy tests can occur and may result in a misdiagnosis. Hyperinsulinemia in polycystic ovary syndrome correlates with increased cardiovascular risk independent of obesity.

  • Outflow tract obstruction.

  • Mayo Clin Proc.

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  • Citing articles via Web of Science J Pediatr Adolesc Gynecol.

  • Amenorrhea: Evaluation and Treatment.

Menses may return after a modest increase in caloric intake or a decrease in athletic training. Other rpimary causes of pure gonadal dysgenesis can occur with a 46,XY or XX karyotype. Information from references 6 and No sponsor or advertiser has participated in, approved or paid for the content provided by Decision Support in Medicine LLC. Pelvic radiation. Bronchogenic e. Address correspondence to Tarannum Master-Hunter, M.

Peak growth 11 to We want you to take advantage of everything Cancer Therapy Advisor has to offer. Speroff L, Fritz MA. A family history significant for delayed puberty should prompt a genetic work-up. If tumor is excluded as the cause, medications e. Central nervous system tumor.

Although diagnosis of hypogonadotropic hypogonadism is fairly simple, elucidation of the cause of disease in patients is challenging. Diagnosis and management of pituitary tumors: recent advances. Medroxyprogesterone acetate Provera. Traggiai C, Stanhope R.

Get Permissions. Login Register. False-negative results occur in urine that is too dilute. Instead, they interfere with one antibody and cause a false-negative result. Treatment of microadenomas should focus on management of infertility, galactorrhea, and breast discomfort.

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The authors thank Barbara S. Treatment of microadenomas should focus on management of infertility, galactorrhea, and breast discomfort. Best Value! Kallmann syndrome, which is associated with anosmia, also can cause hypogonadotropic hypogonadism. Conjugated equine estrogen Premarin.

Magnetic resonance imaging scan of the hypothalamic-pituitary region was normal. On average, menopause occurs at 50 years of age and is caused by ovarian follicle depletion. In addition, what follow-up tests might be useful? Google Scholar PubMed.

If the patient has a normal uterus, outflow tract obstruction should amenorthea considered. Ovarian dermoid cyst. TABLE 2 History and Physical Examination Findings Associated with Amenorrhea Findings Associations Patient history Exercise, weight loss, current or previous hypgoonadism illness, illicit drug use Hypothalamic amenorrhea Menarche and menstrual history Primary versus secondary amenorrhea Prescription drug use Multiple, depending on medication Previous central nervous system chemotherapy or radiation Hypothalamic amenorrhea Previous pelvic radiation Premature ovarian failure Psychosocial stressors; nutritional and exercise history Anorexia or bulimia nervosa Sexual activity Pregnancy Family history Genetic defects Multiple causes of primary amenorrhea Pubic hair pattern Androgen insensitivity syndrome Infertility Multiple Menarche and menstrual history mother and sisters Constitutional delay of growth and puberty Pubertal history e. Screening of autosomal gene deletions in patients with hypogonadotropic hypogonadism using multiplex ligation-dependent probe amplification: detection of a hemizygosis for the fibroblast growth factor receptor 1. The risk of amenorrhea is lower with subclinical hypothyroidism than with overt disease.

Patients who have not developed secondary sex characteristics, especially the absence of breast development, and have not established periodic menstruation by hypogonadotropix years of age should also be worked up for primary amenorrhea. On average, menopause occurs at 50 years of age and is caused by ovarian follicle depletion. In patients with primary amenorrhea, the presence or absence of sexual development should direct the evaluation. Normal and abnormal sexual development. Patients with normal TSH and prolactin should be evaluated for gonadotropic function by measuring luteinizing hormone LH and follicle stimulating hormone FSH.

J Adolesc Health. Primary amenorrhea hypogonadotropic hypogonadism and Hypoogonadism responses were similar in both sets of women. HH is typically characterized by low circulating sexual steroids associated with low or inappropriately normal gonadotropin levels. Chronic renal insufficiency. If testes are present, they should be removed because of the high risk of malignant transformation after puberty.

  • Indeed, the GnRH test provides no extra diagnostic information relative to baseline gonadotropin levels. Close more info about Primary Amenorrhea due to Hypothalamic disease.

  • Progestogen challenge test. An elevated prolactin result should prompt a physician to perform an MRI in search of a pituitary adenoma.

  • The hCG doses should be titrated based on testosterone levels, targeting middle normal values.

  • Delayed puberty.

  • Related articles in Web of Science Google Scholar. Evidence that cells expressing luteinizing hormone-releasing hormone mRNA in the mouse are derived from progenitor cells in the olfactory placode.

Pituitary adenoma. Recombinant human leptin in women priimary hypothalamic amenorrhea. A person viewing it online may make one printout of the material and may use that printout only for his or her personal, non-commercial reference. Several estrogen-containing drugs, such as birth control pills and hormone replacements, may interfere with the assays causing falsely elevated results. Estradiol Estrace. Low estrogen concentrations hypogonadism are expected in patients with primary hypothalamic amenorrhea. First morning specimens are recommended.

This type of phenotypic heterogeneity may be ascribed to environmental or epigenetic effects. Close mobile search navigation Article Navigation. Patients who have not developed secondary sex characteristics, especially the absence of breast development, and have not established periodic menstruation by 13 years of age should also be worked up for primary amenorrhea. Albanese A, Stanhope R.

If a patient has no secondary sexual characteristics and no menarche, primary amenorrhea can be diagnosed as early as 14 years of age. A patient with markedly elevated prolactin levels, galactorrhea, headaches, or visual disturbances should receive imaging tests to rule out a pituitary tumor. In these tumors, measurement of free alpha subunit may assist in monitoring therapy. Physical examination. Amenorrhea: Evaluation and Treatment.

American College of Obstetricians and Gynecologists. Hypogonadidm micronized. Bone mineral changes in primary amenorrhea hypogonadotropic hypogonadism women with hypothalamic amenorrhea treated with oral contraceptives, medroxyprogesterone, or placebo over 12 months. Adult characteristics 13 to No sponsor or advertiser has participated in, approved or paid for the content provided by Decision Support in Medicine LLC. Androgen insensitivity syndrome. Best Value!

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D, and Ana Claudia Latronico, M. Liver failure. Women with premature ovarian failure have an increased risk of osteoporosis amneorrhea heart disease. Drugs that can reversibly suppress sex steroid levels include opiates, glucocorticoid, and psychotropic agents such as phenothiazines. J Bone Miner Res. In particular, does your patient take any medications — OTC drugs or Herbals — that might affect the lab results?

Does age at natural menopause affect mortality from ischemic heart disease?. Receive exclusive offers and updates from Oxford Primary amenorrhea hypogonadotropic hypogonadism. Serum testosterone normal: 20 to 80 ng per dL [0. The Endocrine Society has achieved Accreditation with Commendation. Normal MRI indicates a hypothalamic cause of amenorrhea. If a patient with amenorrhea has breast development and minimal or no pubic hair, the usual diagnosis is androgen insensitivity syndrome i. Patients younger than 30 years should receive a karyotype analysis to rule out the presence of a Y chromosome and the need for removal of gonadal tissue.

Statistical study]. Early induction of spermatogenesis may reduce the time required for appearance of sperm and the need for prolonged cycles of gonadotropin treatment in adult life. Young women with HH are at risk for bone loss and fracture.

Endocrinol Metab Clin North Hypogonaxism. A comparison of cabergoline and bromocriptine in the treatment of hyperprolactinemic amenorrhea. The epidemiology of polycystic ovary syndrome. Thus, the laboratory work-up should include measurement of human chorionic gonadotropin hCG to rule out pregnancy. Patients with a detectable uterus should be evaluated for hypothyroidism and prolactinemia. Exercise, weight loss, current or previous chronic illness, illicit drug use. Arch Dis Child.

More in Pubmed Citation Related Articles. Menarche and menstrual history mother and sisters. The condition often is caused by excessive weight loss, exercise, or stress. Next: Arrhythmogenic Right Ventricular Dyplasia.

Clomiphene and oral contraceptives may reduce estrogen concentrations. J Clin Endocrinol Metab. A person viewing it online may make one printout of the material and may use that printout only for his or her personal, non-commercial reference. Prevalence and associated disease risks.

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