Rps138 Slide Amenorrhea

7/30/2019 Rps138 Slide Amenorrhea

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AMENORRHEAAMENORRHEAAMENORRHEAAMENORRHEA

Prof.DR.Dr.H.M.Thamrin Tan un , S .OG(K)Dr.M.Rusda Harahap, Sp.OG


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e n ons

r mary amenorr ea

Failure of menarche to occur when expected in.

No menarche by age 16 years with signs of pubertal

development.

No onset of pubertal development by age 14 years.

Secondary amenorrhea

Absence of menstruation for 3 or more months in apreviously menstruating women of reproductive

.


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- -Ovary-uterus I nt eract ion

Neural cont rol Chemical cont rol

( -) (+ ) ( -)

H othalamus

Gn-RH

Ant . pit uit ary ?FSH, LH

Ovaries ProgesteroneEstrogen

Uterus

Menses


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Inadequate hormonal stimulation of the endomeriumnovu a ory amenorr ea

- Euestrogenic

- ypoes rogen c

Ovulatory amenorrhea

- - -

- XY-Females ( e.g T.F.S)

- Dama ed endometrium e. Ashermans s ndrome


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Amenorrhea

Normal androgens Hypothalamic-pituitary High androgens PCOS

loss or gain, exercise,pseudocyesis)

H er rolactinemia

tumour

Cushings syndrome

Feminizing ovarian tumour Non-gonadal endocrine

hyperplasia (late onset)

, Systemic illness


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AN APPROACH FOR DIAGNOSIS

HISTORY

ULTRASOUND EXAMINATION

Exclude Pregnancy

Exclude Cryptomenorrhea


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ryptomenorr ea

Outflow obstruction to menstrual blood- Imperforate hymen

- ransverse ag na sep um w unc on ng u erus- Isolated Vaginal agenesis with functioning uterus

-

- I nterm it t ent abdominal pain

- oss e cu y w m c ur on- Possible low er abdominal sw ell ing

- Bulging blu ish membrane at t he int roit us or absent

vagina (only dimple)


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I mperforate hymen


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Once Pregnancy and cryptomenorrhea are

The pat ient is a bioassay for

Four categories of pat ients are ident if ied

1. Amenorrhea w it h absent or poorsecondary sex Characters

. menorr ea w norma rysex characters

. androgen excess

.and vagina


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AMENORRHEAAbsent or poor secondary sexCharacteristics

FSH Serum level

Low / normal High

H o onadot ro ic Gonadal

hypogonadim dysgenesis


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Norm al secondary sex Character ist ics

- FSH, LH, Prolactin, TSH- Provera 10 mg PO daily

x 5 days

+ Bleeding No bleeingProlactinTSH

- Mild hypothalamicdysfunction

Work-up(Endocrinologist)

- PCO ( LH/FSH) ev ew resu

And history (next sl ide)


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Hypothalamic-pituitary

Failure

Ovarian

failure

If < 25 yrs or primaryhead CT- scan or MRI

amenorrhea karyoptypeIf < 35 yrs R/O

autoimmune disease

- evere ypo a am cdysfunction

?? Ovar ian biopsy


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Utero-vaginal absence

Karyotype

46-XX46-XY

MullerianAgenesis

AndogenInsenit ivi ty

. Gonadal regressioon

. Testocular enzyme

(MRKH syndrom e)(TSF syndrome)defenciecy. Leydig cell agenisis

Normal breast sNormal breast s

& sexual haira sen sexuahair

sen reas s& sexual hair


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Normal FSH, LH; -ve bleedinghistory is suggestive of amenorrheatrumatica

Ashermans syndrome History of pregnancy associated D&C

Rarely after CS , myomectomy T.B

endometritis, bilharzia agnos s : or ys erescopy

Treatment : lysis of adhesions; D&C or

catheter)

ome w prescr e a cyc e o s rogen an

Progesterone challenge Before HSG or Hysterescopy


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Ashermans syndrome


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Signs of androgen excess

Testosterone, DHEAS, FSH, and LH

DHEAS 500-700 mug/dLDHEAS >700 mug/dLTEST. >200 ng/dL

Serum 17-OH

Progesterone level

U/S ? MRI or CT

Late CAHrenahyperfunctionOr adrenal

tumor

Lower elevations PCOS (High LH / FSH)


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Amenorrhea

PRIMARY AMENORRHEA

SECONDARY AMENORRHEA

.

. Hypogonadotrophic 34%

Hypogonadism.

.

. Premature ovarian failure 29%

. Weight related amenorrhoea 19%. PCOS 17%

. Congenital lesions

(other than dysgenesis) 4%

. Hyperprolactinaemia 14%

. Exercise related amenorrhoea 2%

. Hypopituitarism 2%

. Hypopituitarism 3%

. Hyperprolactinaemia 3%

.


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Gonadal dysgeneis

Chromosomally incompetent- Classic turners syndrome (45XO)

- urner variants (45XO/46XX),(46X-abnormal X)

- Mixed gonadal dygenesis (45XO/46XY) romosoma y compe en

- 46XX (Pure gonadal dysgeneis)

-


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Gonadal dysgenesis

Turners Variant Dysgenesis Dysgenesis

phenotype Female Female Female Ambiguous

Gonad Streak Streak Streak - Streak

- Testes

Hight Short - Short

- Normal

Tall Short

Classical Nil

stigmata

karyotype XO XX/XO or 46-XX(Pure) XO/XYa norma 46-XY (Swyer)


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Turners syndrome

.

Associated abnormalities, webbed neck,coarctation ofthe aorta,high-arched pallate, cubitus valgus, broads e - e c es w w e y space n pp es, owhairline on the neck, short metacarpal bones and

renal anomalies. High FSH and LH levels.

Bilateral streaked gonads.

- ,

- 20% mosaic forms (46XX/45X0)

Treatment: HRT


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None-dysgenesis ovarian failure

Steroidogenic enzyme defects (17-hydroxylase) Ovarian resistance s ndrome

Autoimmune oophoritis

Postinfection e . Mum s

Postoopherectomy

Postchemotherapy


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Premat ure ovar ian failure

Serum estradiol < 50 pg/ml and FSH > 40 IU/ml onrepeated occasions

o secon ary amenorr ea

Few cases reported, where high dose estrogen or

Sometimes immuno therapy may reverse autoimmueovarian failure

Rarely spont. ovulation (resistant ovaries) Treatment: HRT (osteoporosis, atherogenesis)


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Polycyst ic ovary syndrome

The most common cause of chronic anovulation Hyperandrogenism ; LH/FSH ratio

Insulin resitance is a major biochemical feature

(

blood insulin level

hyperandrogenism ) ong erm r s s: es y, rsu sm, n er y,type 2 diabetes, dyslipidemia, cardiovasularrisks endometrial h er lassia and cancer

Treatment depends on the needs of the patientand preventing long term health problems


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Hypogonadism

Normal hight

Normal external and internalni l r n inf n il

Low FSH and LH

MRI to R/O intra-cranial pathology. 30-40% anosmia (kallmanns

syndrome)

Sometimes constitutional dela

Treat according to the cause (HRT),potentially fertile.


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Const it ut ional pubertal delay

Under stature and delayedbone age

( X-ray Wrist joint)

Positive family history

follow up

Prognosis is good

(late developer) No drug therapy is required

Reassurance ? HRT


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Sheehans syndrome

Pituitary inability to secrete gonadotropins Pituitar necrosis followin massive obstetric

hemorrhage is most common cause in women

Diagnosis : History and E2,FSH,LH+ other pituitary deficiencies (MPS test)

reatment :

Replacement of deficient hormones


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-Anorexia Nervosa

1o or 2o Amenorrhea is often first sign

A body mass index (BMI)


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-amenorrhoea

Common in women who participatein sports (e.g. competitive athletes,

Eating disorders have a higher

prevalence in female athletes than-

Hypothalamic disorder caused byabnormal gonadotrophin-releasing

ormone pu sa y, resu ng nimpaired gonadotrophin levels,particularly LH, and subsequentlyow oes rogen eve s


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amenorrhea

Post-pill amenorrhea is not an entity Depot medroxyprogesterone acetate

Up to 80 % of women will have amenorrheaafter 1 year of use. It is reversible

A minority of women taking the progestogen-onl ill ma have reversible lon term

amenorrhoea due to complete suppression ofovulation


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hyperplasia

Autosomal recessive trait Most common form is due to 21-

y roxy ase e c ency

Mild forms Closely resemble PCO

severe androgen excess

High 17-OH-progesterone blood

level Treatment : cortisol replacement


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Cushings syndrome

Clinical suspicion : Hirsutism,truncal obesity, purple striae, BP

dexamethasone suppression test

(1 mg PO 11 pm ) and obtaineserum cor so eve a am :

< 5 g/ dl excludes cushings

24 hours total urine free cortisol

level to confirm diagnosis 2 forms ; adrenal tumour or ACTH

site)


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-Mayer-Roki t ansky-Kuster-Hauser syndrome

15% of 1ry amenorrhea

Normal breasts and Sexual Hairdevelo ment & Normal lookin externalfemale genitalia

Normal female range testosterone level

ovaries

Karyotype 46-XX

- ,anomalies

Treatment : STERILE ? Vaginal creation


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Test icular feminizat ion syndrome

X-linked trait

Absent cytosol receptors

Normal looking female external

genitalia Absent uterus and upper vagina

Karyotype 46, XY

Treatment : gonadectomy afterpuberty + HRT

? Vagina creation i atation VVaginoplasty )


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of Amenorrhea

. Attempts to restore ovulatory function

. If this is not possible HRT (oestrogen and

progesterone) is given to hypo-estrogenic

amenorrheic women (to prevent osteoporosis; atherogenesis). Periodic progestogen should be taken by euestrogenic

amenorrheic women v i i l

. If Y chromosome is present gonadectomy is indicated

. any cases requ re requen re-eva ua on


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Hormonal t reatmentPr imary Amenorrhea w it h absentsecondary sexual character ist ics

To achieve pubertal developmentremar n mg - + provera mg -

X 3 months; 2.5mg premarin X 3 months and

1.25mg premarin X 3 months

Maintenance therapy

0.625mg premarin + provera OR ready HRT


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ummary -

complex, a carefully conducted physical examination with thehistory, and Looking to the patient as a bioassay for

endocrine abnormalities, should permit the clinician to narrow

the diagnostic possibilities and an accurate diagnosis can beo a ne qu c y.

Management aims at restoring ovulatory cycles if possible,

protect endometrium from unopposed estrogen.

- .


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