AMENOREA

PRIMER
A.A.N ANANTASIKA
 BATASAN
SPEROFF (2005)
I. GADIS USIA 14 TH TANDA SEKS SEKUNDER
(-) & BLM MENARKE
II. GADIS USIA 16 TH TANDA SEKS SEKUNDER
POS TETAPI BELUM MENARKE
 UMUR BUAH HAID JENIS
(TH) DADA
14 Ө Ө PRIMER

16 (+) Ө PRIMER

3 SIKLUS Ө
PASKA SEKUNDER
MENARCHE 6 BULAN Ө
RATA-RATA UMUR GADIS SAAT
MULAI PUBERTAS

-AWAL PERTUMBUHAN BUAH DADA 10,8  1,10

-RAMBUT KEMALUAN 11,0  1,21

-MENARCHE 12,9  1,20

MASHCHAK
GROUP BUAH UTERUS KETERANGAN
DADA
I Ө  SENTRAL
PERIFER

II  Ө RKH
TFS

KEL SEKS
III Ө Ө KROMOSOM

IV   EVALUASI SPT
AMENORE
SEKUNDER
PERTUMBUHAN BUAH DADA
MERUPAKAN PETANDA
ADANYA

ESTROGEN
KLASIFIKASI SEKS SEKUNDER TANNER
 TANNER STAGING
BREAST PUBIC HAIR
STAGE 1 ELEVATION OF PAPILA ONLY NO PUBIC HAIR
(PREPUBERTAL)

STAGE 2 ELEVATION OF BREAST AND SPARSE,LONG,PIGMENTED

PAPILA AS SMALL MOUND,
HAIRLY CHIEFLY ALONG LABIA
AREOLA DIAMETER ENLARGED
MAJORA,MEDIAN AGE 10,5 YRS
MEDIAN AGE:9,8 YRS
STAGE 3 FURTHER ENLARGEMENT DARK,COARSE,CURLED HAIR
WITHOUT SEPARATION OF SPARSELY SPREAD OVERMONS
BREAST AND AREOLA
MEDIAN AGE 11,4 YRS
MEDIAN AGE 11,2 YRS
STAGE 4 SECONDARY MOUND OF ADULT TIPE HAIR,ABUNDANT
AREOLA AND PAPILA ABOVE THE
BUT LIMITED TO THE MONS
BREAST
MEDIAN AGE 12,1 YRS MEDIAN AGE 12,0 YRS
STAGE 5 RECESSION OF AREOLA ADULT TYPE SPREAD IN
TO CONTOUR OF BREAST QUANTITY AND DISTRIBUTION
MEDIAN AGE 14,6 YRS MEDIAN AGE 13,7 YRS
MASHCHAK
GROUP BUAH UTERUS KETERANGAN
DADA
I Ө  SENTRAL
PERIFER

II  Ө RKH
TFS

KEL SEKS
III Ө Ө KROMOSOM

IV   EVALUASI SPT
AMENORE
SEKUNDER
 GROUP I
(BUAH DADA Ө UTERUS )

PROD ESTROG
NEGATIF

TINGGI PX FSH SERUM RENDAH

B. HIPOTAL-PITUITARI
GAGAL
A. OVARIUM GAGAL
HIPOG-HIPOG
HIPER - HIPOG
 A. OVARIUM GAGAL
HIPER-HIPOG

E ↓↓↓

KELAINAN ORGAN KELAINAN

(OVARIUM) SINTESA E (ENZYM)
 A. OVARIUM GAGAL
HIPER-HIPOG

KEL KROMOSOM

ANDROGENIK **

NON ANDROGENIK
-45 XO (S. TURNER) -45 X/46 XY
-46 ABNORMAL X DELESI -45 X/46 X (Yq)
LENGAN PENDEK/PANJ. -45 X (TESTISCULAR
-MOSAIK (X/XX,X/XX/XXX, DETERMINAN POSITION)
X/ XY)
-46 XX/ ATAU 46 XY (MURNI
GONADAL DISGENESIS)
-46 XX DG DEF 17 HIDROK ** MESKIPUN TIDAK DITEMUKAN Y KROM
SILASE BILA ADA TANDA-TANDA HIPERANDROGEN
INDIKASI GONADEKTOMI
 SINDROMA TURNER

KLINIS KEL ORGAN LAIN AUTO IMUN DISEASES

-PENDEK -RECCURENT OTITIS

-WEBBING OF THE NECK MEDIA -HASHIMOTO’S
-JARAK NIPLE JAUH -JANTUNG
-UDEMA KAKII & TGN -GINJAL THYROIDITIS
-KUBITUS VALGUS -TULI -ADDISON’S DIS
-MULTIPLE PIGMENTED -OSTEOPOROSIS -ALOPECIA
NEVI -RESIST INSULIN RINGAN
-VITILIGO
 TERAPI SINDROMA TURNER

PERTUMBUHAN FERTILITAS

CONTINOUS DAILY LOW DOSE ANDROGEN

PRESENT: OOCYTE
CESSATION OF LINEAR GROUP DONATION BY IVF-ET

FUTURE:CRYOPESERVATION
OF FUNCTIONAL OOCYTE
ESTROGEN ON DAYS 1-25 OF EACH MONTHS OBTAINED IN INFANCY
PROGESTIN ON DAYS 14-25 OF EACH MONTHS
 A. OVARIUM GAGAL
HIPER-HIPOG

E ↓↓↓

KELAINAN ORGAN KELAINAN

(OVARIUM) SINTESA E (ENZYM)
CHOLESTEROL
STEROIDOGENESIS
P450scc

3
CORTICO
PREGNENOLON  DOC ALDOST.

11
PROGEST.

21 HYDROXYLASE
HYDROXY STEROID DEHYDROGENASE COST.


HYDROXYLASE
17  HYDROXYLASE
11 
HSD
17 OH DESOXY
17 OH PROGEST CORTISOL CORTISON
PREGNENOLONE CORTISOL

17,20 DESMOLASE

5

DEHYDROGENASE

DEHYD.

REDUCTASE
17 OH STEROID

DHEA ANDROST TESTOSTERONE

TESTOST.

AROMATASI

ESTRONE ESTRADIOL
DEF.17  HIDROXYLASE

CORTISONE 

ACTH ↑
DEFISIENSI 17  HIDROXILASE

1. GENITALIA EKSTERNA WANITA

2. RETENSI NATRIUM DAN HIPOKALEMIA

3. HIPERTENSI
 TEST ACTH

DARAH 60 MENIT
BASAL
PERIKSA
DARAH

COSYNTROPIN
0,25 MG IV INTERVAL
30-60 DTK

8-9 PAGI DEF 17  HIDROXYLASE,BILA:

(PUASA SEMALAM)
-PROGESTERON ↑↑
-17  HIDROKSI PROGESTERON ↑ SDKT/TETAP
 BLOOD PRESSURE MEASUREMENT IN DIAGNOSIS
GROUP I PRIMARY AMENORHEA

SERUM FSH LEVEL

ELEVATED

HIPERGONADOTROPIC
HIPOGANADISME

BLOOD PRESSURE MEASUREMENT

HIPERTENSION

NORMAL BLOOD PRESSURE

-17  HIDROX DEF

GONADAL DISGENESIS -46 XX
45,X
46,X/ ABNORMAL X
MOSAICISME
KARYOTIPE PURE GONADAL DISGENESIS
45 X/ 46 XY
45 X/TESTICULER
DETERMINANT POSITION
 GROUP I
(BUAH DADA Ө UTERUS )

PROD ESTROG
NEGATIF

TINGGI PX FSH SERUM RENDAH

B. HIPOTAL-PITUITARI
GAGAL
A. OVARIUM GAGAL
HIPOG-HIPOG
HIPER - HIPOG
 CT OR MRI IN DIAGNOSIS OF GROUP I
PRIMARY AMENORRHEA

SERUM FSH

LOW/NORMAL

HIPOGONADOTROPIC
HIPOGONADISM

CT OR MRI SELLA TURSICA

NO LESION LESION

GnRH STIMULATION TEST

NORMAL,FSH,LH ABSENT
RESPONSE RESPONSE

HIPOTHALAMIC PITUITARY
FAILURE FAILURE
 TEST GnRH
100  G GnRH IV
BOLUS 30”

100 G GnRH/HR IM 3O’ 60’

1 MINGGU
DRH PX PX
BASAL LH FSH
2X/15
MNT

KEL HIPOTHALAMUS -30’ LH ↑↑

BILA -60’ FSH ↑↑ PITUITARY BAIK
 GROUP I
(BUAH DADA Ө UTERUS )

PROD ESTROG
NEGATIF

TINGGI PX FSH SERUM RENDAH

B. HIPOTAL-PITUITARI
GAGAL
A. OVARIUM GAGAL
HIPOG-HIPOG
HIPER - HIPOG
 PENATALAKSANAAN AMENOREA PRIMER GROUP 1
BUAH DADA -, UTERUS +, (SENTRAL/PERIFER)

TINGGI FSH
RENDAH/
HYPER-HYPOG
NORMAL

SENTRAL
TENSI PERIFER (E )
(PITUITARI- HYPOTAL)
OV. GAGAL TENSI N

TEST PROVOKASI CT SCAN TEST PROVOKASI

GGN. SINTESA E
ACTH OV GGL GnRH
CC
KEL + KEL -
KARYOTYPING
-
PROGESTERON NON
17 OH PROGEST SDKT ANDROGENIK
ANDROGENIK
HIPOTAL +
NAIK
XO
X/XX/XXX ADA KOMP Y /&
TANDA ANDROGEN PITUITARI
X/XXX
GGN SINTESA E
DEF. 17  HYDROGENASE
AWAS GGN PERTBHN PROLAKTI
KEL ORGAN LAIN SEKS SEKUNDER FERTILITAS NOMA LAIN
AUTOIMUN GONADEKTOMI
CORTISON HRT
ANDROGEN (TB) OOSIT DONOR BROMO
HRT IVF BEDAH
SP. PERTUMBHN
KRIPTIN
HRT OPTIMAL (3 BLN TUMBUH
LAGI)
 COMPLETE DIAGNOSTIC EVALUTION OF WOMEN WITH GROUP I PRIMARY AMENORRHEA

LOW OR NORMAL SERUM FSH

ELEVATED
LEVEL

HYPERGONADOTROPIC
HYPOGONADOTROPIC HYPOGONADISM
HYPOGONADISM

BLOOD PRESSURE
CT OR MRI, PROLAKTIN
TSH
NORMAL BLOOD
PRESSURE HYPERTENSION

NO LESION LESION
GYNADAL
17HYDROX
DYSGENESIS
DEFICIENCY
46, XX
GnRH STIMULATION TEST
KARYOTYPING

NORMAL FSH, LH ABSENT 45,X 45,X

RESPONSE RESPONSE 46,X/ABNORMAL X TESTICULAR
MOSAICISM
PURE GONADAL, DETERMINANT
HYPOTHALAMIC PITUITARI
45,X/ 46 XY POSITION
FAILURE FAILURE
45,X/45,Xi (Yq)
MASHCHAK
GROUP BUAH UTERUS KETERANGAN
DADA
I Ө  SENTRAL
PERIFER

II  Ө RKH
TFS

KEL SEKS
III Ө Ө KROMOSOM

IV   EVALUASI SPT
AMENORE
SEKUNDER
 GUP II AMENOREA
(BUAH DADA POS,UTERUS NEG)

ESTROG
POS

RKH GGAN TFS

PERTUMB
ORGAN
REPROD

RKH : MAYER ROKITANSKY KUSTER HAUSER SYNDROME

TFS : TESTISCULAR FEMINIZATION SYNDROME
 KLASIFIKASI KELAINAN
PERTUMBUHAN DUKTUS MULLER

I. AGENESIS (RKH)
II. GANGGUAN FUSI VERTIKAL
A. OBSTRUKSI
B. NON OBSTRUKSI
III. GANGGUAN FUSI LATERAL
A. OBSTRUKSI
B. NON OBSTRUKSI
 RKH

DASARNYA GENOTIPE WANITA

AGENESIS DUKTUS MULLER
(OVARIUM NORMAL)

KLINIS

•H – P – O  BAIK  OVULASI POS

•TESTOSTERON “N” WANITA
•GANGGUAN PADA GENITALIA : VAGINA DAN UTERUS
 GUP II AMENOREA
(BUAH DADA POS,UTERUS NEG)

ESTROG
POS

RKH GGAN TFS

PERTUMB
ORGAN
REPROD

RKH : MAYER ROKITANSKY KUSTER HAUSER SYNDROME

TFS : TESTISCULAR FEMINIZATION SYNDROME
 NORMAL DEVELOPMENT OF INTERNAL AND EXTERNAL GENITAL

UNDIFFERENTIATED GONAD

XX OVARIAN KARYOTIPE

XY
DEVELOPMENT

WOLFIAN DUCT REGRESION TESTICULAR

+ DEVELOPMENT

MULLERIAN DEVELOPMENT OF MIS

OVIDUCTS AND UTERUS TESTOST. AND DEHYDROTEST

+
T DHT
FEMALE EXTERNAL GENITAL

WOLFIAN OV. DEV. OF

MALE EXT
DEV.
GENITAL

MULLERIAN DUCT.
SEMINAL LAB SCROTAL
REGRESSION VES. AND FUSION+
PROSTAT PHALLIC DEV.
 XY

NORMAL TIDAK NORMAL

(GGN PERTUMBUHAN)

TFS

GENOTIPE: LAKI
PENOTPE: WANITA
 PERBEDAAN RKH DAN TFS
PEMERIKSAAN RKH TFS
KARYOTIPING 46 XX 46 XY

HEREDITER ? MATERNAL X LINKED

RECESSIVE 25% ANAK
TERKENA 25% CARIER
RAMBUT SEKS “N” WANITA NEG/SEDIKIT

TESTOSTERON “N” WANITA “N” / ↑ WANITA

KELAINAN LAIN SERING JARANG

KEGANASAN GONAD NORMAL 5% KEMUNGKINAN

GANAS
 DIFFERENTATION OF THE CONGENITAL ABSENCE OF THE
UTERUS FROM THE ANDROGEN INSENSITIVITY SYNDROME

SERUM TESTOSTERONE &

OBSERVATION OF PUBIC AND
AXILARY HAIR

NORMAL FEMALE TESTOSTERON LEVEL NORMAL MALE TESTOS

AND NORMAL PUBLIC LEVEL AND ABSENT PUBIC
AND AXILARY HAIR AND AXILARY HAIR

ANDROGEN INSENSITIVITY
CONGENITAL ABSENT OF UTERUS (TESTICULAR FEMINIZATION)

CONFIRM OVULATION WITH A BBT OR CONFIRM WITH KARYOTIPE

WEEKLY SERUM PROGESTERONE X 4
 TERAPI AMENOREA GRUP II
BUAH DADA (+) UTERUS (-)

RKH TFS

BEDAH : GONADEKTOMI
VAGINA TIRUAN (PASCA PUBERTAS)

HRT
 PENATALAKSANAAN AMENOREA PRIMER GRUP II,
BUAH DADA +, UTERUS -, (RKH, TFS)

TESTOSTERON TANDA OVULASI

KLINIS/ PMS
TINGGI/ N, RENDAH/ N, BBT
LAKI2 WANITA
RKH USG

+ -
VAGINA TIRUAN

AWAS KEL. BAWAAN LAIN

TFS
GONADEKTOMI
(PASCA PUBERTAS ) HRT
MASHCHAK
GROUP BUAH UTERUS KETERANGAN
DADA
I Ө  SENTRAL
PERIFER

II  Ө RKH
TFS

KEL SEKS
III Ө Ө KROMOSOM

IV   EVALUASI SPT
AMENORE
SEKUNDER
 EVALUATION OF WOMEN WITH GROUP III
PRIMARY AMENORRHEA

KARYOTIPE (46, XY)

ENZYME
DEFICIENCY AGONADISME
 GROUP III AMENORE PRIMER
(BUAH DADA & UTERUS (-) )

A. GANGGUAN ENZIM
DEFISIENSI DARI:
- 17, 20 – DESMOLASE *
- 17  - HIDROKSILASE *
B. TESTICULAR AGONADISM

* KARYOTIPING XY  GONADEKTOMI
 CHOLESTEROL
3  HYDROXY DEHYDROGENASE

MINERALO CORTICOST
20.21 DESMOLASE 11 HYDROXYLASE
2  HYDROXYLASE 18 OXYDASE .
18 HYDROXYLASE
DEOXY 18 hydroksi
5
PRENENOLONE PROGEST CORTICOST
CORTICOST CORTICOST ALDOS

GLUCOCORTICOST
17 HYDROXYLASE

5
17  HYDR 17 HYDROXY DEOXY
CORTISOL
PREGNOL PROGESTERONE CORTISOL

17,20 DESMOLASE

SEX STEROID
DEHYD
5 5
ANDRO
ESTRONE
EPIANDROS STENEDIONE

17  OH STEROID
OXYREDUCTASE
5
17  ANDRO
TESTOSTERONE ESTRADIOL
STENEDIOL
 GROUP III AMENORE PRIMER
(BUAH DADA & UTERUS (-) )

A. GANGGUAN ENZIM
DEFISIENSI DARI:
- 17, 20 – DESMOLASE *
- 17  - HIDROKSILASE *
B. TESTICULAR AGONADISM

* KARYOTIPING XY  GONADEKTOMI
 AMENORE PRIMER GRUP III
AGONADISM

TEORITIS

JAR. TESTISKULAR ADA PADA AWAL PERTUMBUHAN EMBRIO

SISTEM DUCTUS MULLER TERTEKAN

TAPI,SETELAH ITU JAR. TESTISKULAR MENGHILANG

VANISHING TESTES
SYNDROME
THERAPI AMENORE PRIMER GRUP III
(BUAH DADA & UTERUS NEGATIF)

-GONADEKTOMI
-HRT
GRUP IV AMENOREA PRIMER
(BUAH DADA & UTERUS +)

EVALUASI

SAMA DENGAN AMENORE SEKUNDER