Evaluation of Amen or Rhea

8/8/2019 Evaluation of Amen or Rhea

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EVALUATION OFEVALUATION OF

AMENORRHEAAMENORRHEARLBKHLLRLBKHLL-- PGY4PGY4


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D EFINITIOND EFINITIONPrimary amenorrheaPrimary amenorrhea can be diagnosed if a patient hasnormal secondary sexual characteristics but nomenarche by 16 years of age. If a patient has nosecondary sexual characteristics and no menarche,

primary amenorrhea can be diagnosed as early as 14years of age (


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PubertyPuberty1.1. Breast 10.8 y +/Breast 10.8 y +/- - 1.11.1

2.2. Pubic hair 11.0y +/Pubic hair 11.0y +/- - 1.21.23.3. Menarche 12.9y +/Menarche 12.9y +/- - 1.21.2


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D IFFERENTIAL D IAGNOSISD IFFERENTIAL D IAGNOSIS

genetic abnormalitiesgenetic abnormalitiesendocrine disordersendocrine disordersPsychologicalPsychologicalEnvironmentalEnvironmentalStructural anomaliesStructural anomalies

Diagnosis unique to primary amenorrhea include vaginalDiagnosis unique to primary amenorrhea include vaginal

agenesis, androgen insensitivity syndrome, and gonadalagenesis, androgen insensitivity syndrome, and gonadaldysgenesis.The remaining diagnoses should bedysgenesis.The remaining diagnoses should beconsidered in patients with both primary and secondaryconsidered in patients with both primary and secondaryamenorrheaamenorrhea


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ETIOLOGIESETIOLOGIES

Generalized pubertal delayGeneralized pubertal delayC onstitutional delayC onstitutional delayHypergonadotropic hypogonadismHypergonadotropic hypogonadism

Turner syndromeTurner syndromeGonadal dysgenesis with mosaic karyotypeGonadal dysgenesis with mosaic karyotypePure gonadal dysgenesis (Perrault syndrome, Swyer syndrome)Pure gonadal dysgenesis (Perrault syndrome, Swyer syndrome)

GonadotropinGonadotropin- -resistant ovary syndrome: savage syndromeresistant ovary syndrome: savage syndromeFSHFSH--receptor mutationreceptor mutation Acquired causes (eg, high Acquired causes (eg, high- -dose alkylating chemotherapy, pelvic radiation,dose alkylating chemotherapy, pelvic radiation,autoimmune oophoritis)autoimmune oophoritis)C ongenital thymus aplasiaC ongenital thymus aplasia

Hypogonadotropic hypogonadismHypogonadotropic hypogonadismC hronic conditions (eg, starvation, excessive exercise, depression, psychologicalC hronic conditions (eg, starvation, excessive exercise, depression, psychologicalstress, marijuana use, C rohn disease, cystic fibrosis, sickle cell disease,stress, marijuana use, C rohn disease, cystic fibrosis, sickle cell disease,thalassemia major, HIV infection, renal disease, thyroid disease, diabetesthalassemia major, HIV infection, renal disease, thyroid disease, diabetesmellitus, anorexia nervosa)mellitus, anorexia nervosa)SlowSlow--growing C NS tumors (eg, adenomas, craniopharyngiomas, meningiomas,growing C NS tumors (eg, adenomas, craniopharyngiomas, meningiomas,pituitary microadenomas)pituitary microadenomas)Isolated gonadotropin deficiency (kallman sd, midline defects)Isolated gonadotropin deficiency (kallman sd, midline defects) primary amenprimary amen

Acquired miscellaneous disorders (eg, infiltration disorders [sarcoidosis, Acquired miscellaneous disorders (eg, infiltration disorders [sarcoidosis,Langerhans cell histiocytosis, hemochromatosis, syphilis, tuberculomas],Langerhans cell histiocytosis, hemochromatosis, syphilis, tuberculomas],ischemia disorders [caused by trauma, aneurysm, obstruction of the aqueduct of ischemia disorders [caused by trauma, aneurysm, obstruction of the aqueduct of Sylvius] and destruction [concentrated, highSylvius] and destruction [concentrated, high- -dose exposure to radiation])dose exposure to radiation])


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Puberty delayed when no breast development isPuberty delayed when no breast development isevident at 13.5 years, pubic hair is absent at 14evident at 13.5 years, pubic hair is absent at 14years, and menarche is absent at 16 yearsyears, and menarche is absent at 16 yearsMost common cause of delayed puberty isMost common cause of delayed puberty isconstitutional delayconstitutional delay

Another common reason is ovarian failure, also Another common reason is ovarian failure, also

termed hypergonadotropic hypogonadismtermed hypergonadotropic hypogonadismElevated levels of (FSH) and (LH) with lowElevated levels of (FSH) and (LH) with lowestrogenestrogen


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HYPHYP ERER GONADOTROPI C GONADOTROPI C

HYPOGONADISMHYPOGONADISMmost common example of hypergonadotropicmost common example of hypergonadotropichypogonadism is Turner syndrome (45,XO) karyotypehypogonadism is Turner syndrome (45,XO) karyotypewebbed neck, short stature, broad shieldlike chest,webbed neck, short stature, broad shieldlike chest,anomalous auricles, and hypoestrogenemia resulting inanomalous auricles, and hypoestrogenemia resulting insexual immaturitysexual immaturityGonadal dysgenesis is caused by a mosaic karyotypeGonadal dysgenesis is caused by a mosaic karyotypeand streak ovariesand streak ovariesPerrault syndrome = gonadal dysgenesis, a normalPerrault syndrome = gonadal dysgenesis, a normal

karyotype, and neurosensory deafnesskaryotype, and neurosensory deafnessSawyer syndrome = phenotypically immature femaleSawyer syndrome = phenotypically immature femalewith a 46,XY karyotype without testiswith a 46,XY karyotype without testis- -determining factor determining factor on the Y chromosome.on the Y chromosome.GonadotropinGonadotropin- -resistant ovary syndrome, characterizedresistant ovary syndrome, characterized

by FSHby FSH- -resistant ovaries, rare causeresistant ovaries, rare cause


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HYPHYP OOGONADOTROPI C GONADOTROPI C

HYPOGONADISMHYPOGONADISMFSH and LH levels are lowFSH and LH levels are lowC hronic illnessC hronic illness malabsorption andmalabsorption and

malnutritionmalnutritionC NS Tumors compress the portal vesselsC NS Tumors compress the portal vessels

affectaffect flow of GnRH from theflow of GnRH from thehypothalamus to the pituitary glandhypothalamus to the pituitary glandProlactinomas are most common pituitaryProlactinomas are most common pituitarytumorstumors


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ETIOLOGIES

NORMAL PUBERTYNORMAL PUBERTY Associated with hyperandrogenicity (eg, P C O Associated with hyperandrogenicity (eg, P C Osyndrome, latesyndrome, late- -onset 21onset 21- -hydroxylase deficiencyhydroxylase deficiency[nonclassic congenital adrenal hyperplasia],[nonclassic congenital adrenal hyperplasia],immaturity of the hypothalamicimmaturity of the hypothalamic- -pituitarypituitary--ovarianovarianaxis, C ushing disease, androgenaxis, C ushing disease, androgen- -producingproducingovarian or adrenal tumors, ovarian stromalovarian or adrenal tumors, ovarian stromalhypertrophy)hypertrophy)

Anovulation with absence of hirsutism or Anovulation with absence of hirsutism or virilization (eg, immaturity of the hypothalamicvirilization (eg, immaturity of the hypothalamic- -pituitarypituitary--ovarian axis, pregnancy)ovarian axis, pregnancy)Hypergonadotropic hypogonadism (eg, ovarianHypergonadotropic hypogonadism (eg, ovarianfailure, highfailure, high- -dose alkylating chemotherapy,dose alkylating chemotherapy,pelvic radiation, autoimmune oophoritis)pelvic radiation, autoimmune oophoritis)


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ASSO C IATED ASSO C IATED

HYPERANDROGENISMHYPERANDROGENISMFrequently associated with hirsutismFrequently associated with hirsutismmost common cause is P C O syndromemost common cause is P C O syndromelatelate--onset 21onset 21- -hydroxylase deficiency, caused byhydroxylase deficiency, caused bymutations in the 21mutations in the 21- -hydroxylase gene resulting inhydroxylase gene resulting inexcessive 17excessive 17- -hydroxyprogesterone levels, also termedhydroxyprogesterone levels, also termednonclassic congenital adrenal hyperplasia and can occur nonclassic congenital adrenal hyperplasia and can occur in 1in 1--10% of women with hirsutism10% of women with hirsutismOther causes of hyperandrogenism include C ushingOther causes of hyperandrogenism include C ushing

disease, ovarian stromal hypertrophy, and androgendisease, ovarian stromal hypertrophy, and androgen- -producing tumors of the ovary and adrenal glandsproducing tumors of the ovary and adrenal glandsExogenous anabolic steroid use should be considered inExogenous anabolic steroid use should be considered inthe differential for hyperandrogenic amenorrheathe differential for hyperandrogenic amenorrhea


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PC OPC Oat least two of the following :

1. oligoovulation or anovulation (usuallymanifested as oligomenorrhea or amenorrhea),

2. elevated levels of circulating androgens(hyperandrogenemia) or clinical manifestationsof androgen excess (hyperandrogenism)

3. polycystic ovaries as defined byultrasonographyLHLH dependent ovarian overproduction of dependent ovarian overproduction of androgensandrogens

Anovulatory cycles may lead to dysfunctionaluterine bleeding and decreased fertility


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C HRONI C ANOVULATIONC HRONI C ANOVULATION

Anovulation remains most common Anovulation remains most commoncause of amenorrhea in the setting of cause of amenorrhea in the setting of nonvirilizationnonvirilizationC hronic anovulation of central originC hronic anovulation of central origin(hypogonadotropic hypogonadism):(hypogonadotropic hypogonadism):

1.1. hypothalamic chronic anovulationhypothalamic chronic anovulation2.2. Hyperprolactinemic chronic anovulationHyperprolactinemic chronic anovulation3.3. hypopituitarismhypopituitarism


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Hypothalamic chronic anovulation: (H C A)Hypothalamic chronic anovulation: (H C A)slowed GnRH release (multifactorial, decreasedslowed GnRH release (multifactorial, decreased

body fat and increased beta endorphins)body fat and increased beta endorphins) Also called functional amenorrhea Also called functional amenorrhea Abrupt cessation of menses without anatomic or Abrupt cessation of menses without anatomic or endocrine abnormalityendocrine abnormality

Anorexia nervosa is most severe form of H C A, Anorexia nervosa is most severe form of H C A,simple weight loss, malnutrition, exercicesimple weight loss, malnutrition, exercice(athletics), emotional stress(athletics), emotional stress

All have low or normal gonadotropins, elevated All have low or normal gonadotropins, elevatedcortisolcortisol


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Hyperprolactinemia :Hyperprolactinemia :15% of amenorrhea cases15% of amenorrhea cases

>75% of women with amenorrhea +>75% of women with amenorrhea +galactorrheagalactorrhea50% have pituitary tumor 50% have pituitary tumor

C onsider primary hypothyroidismC onsider primary hypothyroidism


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Hypopituitarism :Hypopituitarism :C linical presentation depends on age of onsetC linical presentation depends on age of onsetSuspicion if L/O pubic and axillary hair, atrophySuspicion if L/O pubic and axillary hair, atrophyof external genitalia in a women previouslyof external genitalia in a women previouslymenstruatingmenstruatingr/o postpartum pituitary necrosis (Sheehansr/o postpartum pituitary necrosis (Sheehanssyndrome), Failure of lactation is an earlier signsyndrome), Failure of lactation is an earlier signSx : weakness, fatigability, lack of libido, coldSx : weakness, fatigability, lack of libido, coldintolerance, short stature (during childhood)intolerance, short stature (during childhood)

galactorrhea, headaches, or reduced peripheralgalactorrhea, headaches, or reduced peripheralvision could be a sign of intracranial tumor vision could be a sign of intracranial tumor Skin smooth, thin, cool, pale, slow pulse low BPSkin smooth, thin, cool, pale, slow pulse low BPLow Gntropin, low gonadal steroidsLow Gntropin, low gonadal steroids


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P REMATURE OVARRIANP REMATURE OVARRIAN

FAILUREFAILUREamenorrhea, hypoestrogenism, and increasedgonadotropin levels occurring before 40 years of ageand is not always irreversible0.1 percent of women are affected by 30 years of ageand one percent by age 40increased risk of osteoporosis and heart diseasecan be associated with autoimmune endocrine disorderssuch as hypothyroidism, Addisons disease, and

diabetesPatients younger than 30 years karyotype to rule outpresence of a Y chromosome and the need for removalof gonadal tissueOvarian biopsy and antiovarian antibody testing have not

been shown to have clinical benefit


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ETIOLOGIESETIOLOGIES Anomalies of the genital tract Anomalies of the genital tract

Mllerian agenesis (eg, Mayer Mllerian agenesis (eg, Mayer- -RokitanskyRokitansky- -Kuster Kuster--Hauser syndrome) breast present,Hauser syndrome) breast present,uterusuterus- -

Androgen insensitive syndrome Androgen insensitive syndrome- -absent uterusabsent uteruswith normal breast developmentwith normal breast developmentMost common : C ongenital or acquired outflowMost common : C ongenital or acquired outflowobstruction (eg, imperforate hymen, transverseobstruction (eg, imperforate hymen, transversevaginal septum, Asherman syndrome,vaginal septum, Asherman syndrome,

endometrial destruction due to severe infectionendometrial destruction due to severe infectionor surgery)or surgery)Labial fusion seen in femaleLabial fusion seen in femalepseudohermaphrodism ( C AH or exposure topseudohermaphrodism ( C AH or exposure tomaternal androgens in utero)maternal androgens in utero)


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Mayer Mayer--RokitanskyRokitansky- -Hauser Hauser

syndromesyndromeanomaly of the genital tract characterized byanomaly of the genital tract characterized byvaginal agenesisvaginal agenesisUterus is usually absent, vagina is foreshortenedUterus is usually absent, vagina is foreshortened

Ovaries function normally and produce estradiolOvaries function normally and produce estradiol(E2)(E2) breasts normalbreasts normalPubarche also normalPubarche also normalKaryotype 46,XX and is sterileKaryotype 46,XX and is sterile

Accounts for 15% of primary amenorrhea cases Accounts for 15% of primary amenorrhea casesand is second to Turner syndrome as the mostand is second to Turner syndrome as the mostcommon cause of primary amenorrheacommon cause of primary amenorrheaMay require reconstructive surgeryMay require reconstructive surgery


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Spontaneous testicular regression: rare disorder Spontaneous testicular regression: rare disorder of genetic males that results in a femaleof genetic males that results in a femalephenotype with an absent uterus and sec sexphenotype with an absent uterus and sec sex

characteristics (sexual infantism)characteristics (sexual infantism)Enzymatic deficiencies affecting androgenEnzymatic deficiencies affecting androgenproduction can result in maleproduction can result in malepseudohermaphroditespseudohermaphrodites

All disorders that are phenotypically female but All disorders that are phenotypically female butchromosomally male (XY) require that thechromosomally male (XY) require that thegonads be removed to avert cancerous changesgonads be removed to avert cancerous changes


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Asherman syndrome occurs after an Asherman syndrome occurs after anoverzealous curettage of the endometrialoverzealous curettage of the endometriallininglining adhesions or synechiaeadhesions or synechiae

C linically significant infections that destroyC linically significant infections that destroythe endometrial lining can also result inthe endometrial lining can also result inprimary or secondary amenorrhea.primary or secondary amenorrhea.


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HISTORYHISTORYchildhood growth and development,childhood growth and development,including height and weight charts and ageincluding height and weight charts and ageat thelarche and menarcheat thelarche and menarcheage at menarche of mother and sistersage at menarche of mother and sistersduration and flow of menses, cycle days,duration and flow of menses, cycle days,day and date of last menstrual periodday and date of last menstrual period

history of chronic illness, trauma, surgery,history of chronic illness, trauma, surgery,and medications, sexual history,and medications, sexual history,substance use, exercise, diet, home andsubstance use, exercise, diet, home andschool situations, and psychosocial issuesschool situations, and psychosocial issues


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P HYSI C AL EXAMP HYSI C AL EXAMvital signs, including height and weight, and sexualvital signs, including height and weight, and sexualmaturity ratingmaturity rating Anorexia Anorexia - - C achexia, bradycardia, hypotension, andC achexia, bradycardia, hypotension, andhypothermiahypothermia

Pituitary tumor Pituitary tumor - - Funduscopic changes, visual fieldFunduscopic changes, visual fieldimpairment, and cranial nerve signsimpairment, and cranial nerve signsPolycystic ovary (P C O) syndromePolycystic ovary (P C O) syndrome - - Acne, acanthosis Acne, acanthosisnigricans, and obesitynigricans, and obesityInflammatory bowel diseaseInflammatory bowel disease - - Fissure, skin tags, andFissure, skin tags, and

occult bloodoccult bloodGonadal dysgenesis (eg, Turner syndrome)Gonadal dysgenesis (eg, Turner syndrome) - - WebbedWebbedneck, increased carrying angle, and lack of breastneck, increased carrying angle, and lack of breastdevelopmentdevelopment


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GalactorrheaGalactorrhea - - Breast palpationBreast palpationDelayed pubertyDelayed puberty Underdeveloped breast with sparseUnderdeveloped breast with sparsepubic hair pubic hair HyperandrogenismHyperandrogenism - - Pubic hair distribution and excessPubic hair distribution and excess

facial hair facial hair Androgen insensitivity syndrome Androgen insensitivity syndrome - - Absent or sparse Absent or sparseaxillary and pubic hair with breast developmentaxillary and pubic hair with breast development

Adrenal or ovarian tumors Adrenal or ovarian tumors - - C litoromegaly and virilizationC litoromegaly and virilizationPelvic fullnessPelvic fullness - - Pregnancy, ovarian mass, and genitalPregnancy, ovarian mass, and genital

anomaliesanomaliesImperforate hymenImperforate hymen - - Distension or bulging of the externalDistension or bulging of the externalvaginavaginaVaginal agenesis (RokitanskyVaginal agenesis (Rokitansky- -Hauser syndrome)Hauser syndrome)


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LABORATORY EVALUATIONLABORATORY EVALUATIONPRL> 20PRL> 20 primary vs secondaryprimary vs secondaryPRL is elevated in > 1/3 of amenorrheaPRL is elevated in > 1/3 of amenorrheaFSH> 30FSH> 30 mIU/mlmIU/ml ovarian failure, if pt younger ovarian failure, if pt younger

than 30 y do karyotypethan 30 y do karyotypeLH is elevated in P C O, LH/FSH elevatedLH is elevated in P C O, LH/FSH elevatedLH is normal or slightly decreased in femalesLH is normal or slightly decreased in femaleswith HP dysfunctionwith HP dysfunctionLH & FSH < 10 mIU/mlLH & FSH < 10 mIU/ml assess sella turcicaassess sella turcicaTestosterone > 200 ng/ml r/o androgenTestosterone > 200 ng/ml r/o androgensecreting ovarian cancer secreting ovarian cancer DHEAS > 7 mcg/ml r/o adrenal neoDHEAS > 7 mcg/ml r/o adrenal neoDHEAS 5DHEAS 5- -7 mcg/ml consider C AH7 mcg/ml consider C AH


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evaluation of amenorrhea w ith evaluation of amenorrhea w ith d elaye d p ubertyd elaye d p uberty

thyroid functionthyroid functionbone agebone age

luteinizingluteinizing- -hormone (LH)hormone (LH)folliclefollicle--stimulating hormone (FSH)stimulating hormone (FSH)ProlactinProlactin


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TSH elevated and fT4 lowTSH elevated and fT4 low hypothyroidismhypothyroidismBone age delayedBone age delayed constitutional delayconstitutional delayBone age normalBone age normal LH, FSH, and prolactin levelsLH, FSH, and prolactin levels

LH and FSH levels elevatedLH and FSH levels elevated obtain a karyotypeobtain a karyotypeIf karyotype is 45,XO, the cause is gonadal dysgenesisIf karyotype is 45,XO, the cause is gonadal dysgenesis(ie, Turner syndrome)(ie, Turner syndrome)If karyotype is 46,XX, the primary cause is ovarian failureIf karyotype is 46,XX, the primary cause is ovarian failure

Perform an autoimmune workup ( C onsider an etiologyPerform an autoimmune workup ( C onsider an etiology

of autoimmune oophoritis, effects of radiation therapy or of autoimmune oophoritis, effects of radiation therapy or chemotherapy, 17chemotherapy, 17- -alphaalpha- -hydroxylase deficiency, or hydroxylase deficiency, or resistant ovary syndrome)resistant ovary syndrome)


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LH and FSH low or within the referenceLH and FSH low or within the referencerangerange obtain a head MRI.obtain a head MRI.C

heck for pituitary tumor, pituitaryC

heck for pituitary tumor, pituitarydestruction, or hypothalamic diseasedestruction, or hypothalamic diseaseIf prolactin levels are elevated, obtain aIf prolactin levels are elevated, obtain ahead MRI ( r/o secondary causes)head MRI ( r/o secondary causes)


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evaluation of amenorrhea w ith evaluation of amenorrhea w ith normal p ubertynormal p uberty

pregnancy testpregnancy testIf pregnancy test negative, obtain TSH, prolactin, FSH and LH levelsIf pregnancy test negative, obtain TSH, prolactin, FSH and LH levelsWomen < 30 years need to be evaluated by karyotypeWomen < 30 years need to be evaluated by karyotypehyperprolactinemiahyperprolactinemia prolactinoma, C NS tumors and medicationsprolactinoma, C NS tumors and medicationsFSH lowFSH low MRI head (hypothalamic disease, pituitary disease or MRI head (hypothalamic disease, pituitary disease or pituitary tumor vs chronic disease, anorexia nervosa, marijuana or pituitary tumor vs chronic disease, anorexia nervosa, marijuana or cocaine use, and social or psychological stresses)cocaine use, and social or psychological stresses)FSH elevated, ovarian failure is the diagnosisFSH elevated, ovarian failure is the diagnosis karyotypekaryotypekaryotype abnormalkaryotype abnormal consider pure gonadal dysgenesis, such asconsider pure gonadal dysgenesis, such asTurner syndrome or mosaic or mixed gonadal dysgenesisTurner syndrome or mosaic or mixed gonadal dysgenesiskaryotype normal (46 XX)karyotype normal (46 XX) ovarian failure ( premature ovarianovarian failure ( premature ovarianfailure, autoimmune oophoritis, exposure to radiation or failure, autoimmune oophoritis, exposure to radiation or chemotherapy, resistant ovary syndrome)chemotherapy, resistant ovary syndrome)


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If TSH, prolactin, and FSH levels are withinIf TSH, prolactin, and FSH levels are withinreference range, perform a progestin challengereference range, perform a progestin challengetesttestIf withdrawal bleeding occurs, consider If withdrawal bleeding occurs, consider anovulation secondary to P C O syndromeanovulation secondary to P C O syndromeIf no withdrawal bleed occurs, proceed withIf no withdrawal bleed occurs, proceed withestradiol priming followed by a progestinestradiol priming followed by a progestinchallengechallenge

If the challenge does not induce menses,If the challenge does not induce menses,consider Asherman syndrome or outletconsider Asherman syndrome or outletobstructionobstruction


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If hirsutism is present, check testosterone,If hirsutism is present, check testosterone,DHEAS, and 17DHEAS, and 17- -OH progesterone levelOH progesterone levelIf the testosterone and DHEAS levels are withinIf the testosterone and DHEAS levels are withinthe reference range or moderately elevated,the reference range or moderately elevated,perform a progesterone challenge (If withdrawalperform a progesterone challenge (If withdrawalbleeding occurs, the diagnosis is P C OS)bleeding occurs, the diagnosis is P C OS)If the 17If the 17- -OH progesterone level is elevated, theOH progesterone level is elevated, thediagnosis is adult onset adrenal hyperplasiadiagnosis is adult onset adrenal hyperplasiaTestosterone or DHEAS elevated r/o neoplasmTestosterone or DHEAS elevated r/o neoplasm


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evaluation of g enital tract evaluation of g enital tract abnormalitiesabnormalities

Obtain a pelvic sonographyObtain a pelvic sonographyIf the uterus is absent, obtain a karyotypeIf the uterus is absent, obtain a karyotype

If the karyotype is 46,XY, obtain testosterone levels.If the karyotype is 46,XY, obtain testosterone levels.If testosterone levels are within reference range or are highIf testosterone levels are within reference range or are high(male range), the cause is androgen insensitivity(male range), the cause is androgen insensitivityIf testosterone levels are within reference range or are lowIf testosterone levels are within reference range or are low(female range), the cause is testicular regression or gonadal(female range), the cause is testicular regression or gonadalenzyme deficiencyenzyme deficiency

If the karyotype is 46,XX, the cause is mllerianIf the karyotype is 46,XX, the cause is mllerianagenesis (ie, Rokitanskyagenesis (ie, Rokitansky- -Kuster Kuster--Hauser syndrome)Hauser syndrome)


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TREATMENTTREATMENTdirected at correcting the underlying pathologydirected at correcting the underlying pathologyoutflow tract abnormalitiesoutflow tract abnormalities surgerysurgeryDopamine agonists for hyperprolactinemiaDopamine agonists for hyperprolactinemiaOC P for decreased estrogen production (ovarianOC P for decreased estrogen production (ovarianor central)or central)Estrogen required to maintain female secondaryEstrogen required to maintain female secondary

sexual characteristics & bone density (Loss of sexual characteristics & bone density (Loss of menstrual regularity has been associated withmenstrual regularity has been associated withan increased risk of wrist and hip fractures)an increased risk of wrist and hip fractures)


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FunctionalFunctional ameliorate stressful situation,ameliorate stressful situation,decrease exercise, correct weight lossdecrease exercise, correct weight loss+ estroge+ estroge

Gonadotropin therapy or the use of Gonadotropin therapy or the use of pulsatile GnRH therapy is required topulsatile GnRH therapy is required toinduce ovulationinduce ovulation


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Evaluation of Amen or Rhea

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