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 (TH)

14 16

BUAH DADA (+) 3 SIKLUS

HAID

JENIS PRIMER PRIMER

PASKA MENARCHE

SEKUNDER

6 BULAN

RATA-RATA UMUR GADIS SAAT MULAI PUBERTAS

-AWAL PERTUMBUHAN BUAH DADA -RAMBUT KEMALUAN -MENARCHE 10,8 1,10 11,0 1,21 12,9 1,20

MASHCHAK
GROUP BUAH DADA UTERUS
KETERANGAN
SENTRAL PERIFER RKH TFS KEL SEKS KROMOSOM EVALUASI SPT AMENORE SEKUNDER

I II

III
IV

PERTUMBUHAN BUAH DADA MERUPAKAN PETANDA ADANYA

ESTROGEN

KLASIFIKASI SEKS SEKUNDER TANNER

TANNER STAGING
BREAST
STAGE 1 (PREPUBERTAL) STAGE 2 ELEVATION OF PAPILA ONLY

PUBIC HAIR
NO PUBIC HAIR

ELEVATION OF BREAST AND PAPILA AS SMALL MOUND, AREOLA DIAMETER ENLARGED MEDIAN AGE:9,8 YRS

SPARSE,LONG,PIGMENTED HAIRLY CHIEFLY ALONG LABIA MAJORA,MEDIAN AGE 10,5 YRS DARK,COARSE,CURLED HAIR SPARSELY SPREAD OVERMONS MEDIAN AGE 11,4 YRS

STAGE 3

FURTHER ENLARGEMENT WITHOUT SEPARATION OF BREAST AND AREOLA MEDIAN AGE 11,2 YRS

STAGE 4

SECONDARY MOUND OF AREOLA ADULT TIPE HAIR,ABUNDANT AND PAPILA ABOVE THE BREAST BUT LIMITED TO THE MONS MEDIAN AGE 12,1 YRS

MEDIAN AGE 12,0 YRS

STAGE 5

RECESSION OF AREOLA TO CONTOUR OF BREAST MEDIAN AGE 14,6 YRS

ADULT TYPE SPREAD IN QUANTITY AND DISTRIBUTION MEDIAN AGE 13,7 YRS

MASHCHAK
GROUP BUAH DADA UTERUS
KETERANGAN
SENTRAL PERIFER
RKH TFS KEL SEKS KROMOSOM EVALUASI SPT AMENORE SEKUNDER

I
II III IV

GROUP I (BUAH DADA UTERUS )

PROD ESTROG NEGATIF

TINGGI

PX FSH SERUM

RENDAH

A. OVARIUM GAGAL HIPER - HIPOG

B. HIPOTAL-PITUITARI GAGAL HIPOG-HIPOG

A. OVARIUM GAGAL HIPER-HIPOG

KELAINAN ORGAN (OVARIUM)

KELAINAN SINTESA E (ENZYM)

A. OVARIUM GAGAL HIPER-HIPOG

KEL KROMOSOM

ANDROGENIK NON ANDROGENIK -45 XO (S. TURNER) -46 ABNORMAL X DELESI LENGAN PENDEK/PANJ. -MOSAIK (X/XX,X/XX/XXX, X/ XY) -46 XX/ ATAU 46 XY (MURNI GONADAL DISGENESIS) -46 XX DG DEF 17 HIDROK SILASE

**

-45 X/46 XY -45 X/46 X (Yq) -45 X (TESTISCULAR DETERMINAN POSITION)

** MESKIPUN TIDAK DITEMUKAN Y KROM

BILA ADA TANDA-TANDA HIPERANDROGEN INDIKASI GONADEKTOMI

SINDROMA TURNER

KLINIS

KEL ORGAN LAIN

AUTO IMUN DISEASES

-PENDEK -WEBBING OF THE NECK -JARAK NIPLE JAUH -UDEMA KAKII & TGN -KUBITUS VALGUS -MULTIPLE PIGMENTED NEVI

-RECCURENT OTITIS
MEDIA -JANTUNG -GINJAL -TULI -OSTEOPOROSIS -RESIST INSULIN RINGAN

-HASHIMOTOS THYROIDITIS -ADDISONS DIS -ALOPECIA -VITILIGO

TERAPI SINDROMA TURNER

PERTUMBUHAN

FERTILITAS

CONTINOUS DAILY LOW DOSE ANDROGEN

CESSATION OF LINEAR GROUP

PRESENT: OOCYTE DONATION BY IVF-ET FUTURE:CRYOPESERVATION OF FUNCTIONAL OOCYTE OBTAINED IN INFANCY

ESTROGEN ON DAYS 1-25 OF EACH MONTHS PROGESTIN ON DAYS 14-25 OF EACH MONTHS

A. OVARIUM GAGAL HIPER-HIPOG

KELAINAN ORGAN (OVARIUM)

KELAINAN SINTESA E (ENZYM)

CHOLESTEROL P450scc
3 HYDROXY STEROID DEHYDROGENASE
PREGNENOLON
PROGEST.

STEROIDOGENESIS

DOC

CORTICO COST.

ALDOST.

11 HYDROXYLASE

21 HYDROXYLASE

17 HYDROXYLASE
17 OH PREGNENOLONE

17 OH PROGEST

DESOXY CORTISOL

11 HSD
CORTISOL CORTISON

17,20 DESMOLASE DHEA

5 REDUCTASE

17 OH STEROID DEHYDROGENASE

ANDROST

TESTOSTERONE

DEHYD. 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 BASAL 60 MENIT PERIKSA DARAH

COSYNTROPIN
0,25 MG IV INTERVAL 30-60 DTK

8-9 PAGI
(PUASA SEMALAM)

DEF 17 HIDROXYLASE,BILA:

-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

GONADAL DISGENESIS

-17 HIDROX DEF -46 XX 45,X 46,X/ ABNORMAL X MOSAICISME PURE GONADAL DISGENESIS 45 X/ 46 XY 45 X/TESTICULER DETERMINANT POSITION

KARYOTIPE

GROUP I (BUAH DADA UTERUS )

PROD ESTROG NEGATIF

TINGGI

PX FSH SERUM

RENDAH

A. OVARIUM GAGAL HIPER - HIPOG

B. HIPOTAL-PITUITARI GAGAL HIPOG-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 RESPONSE

ABSENT RESPONSE

HIPOTHALAMIC FAILURE

PITUITARY FAILURE

TEST GnRH
100 G GnRH IV BOLUS 30

100 G GnRH/HR IM 1 MINGGU

DRH BASAL
2X/15 MNT

3O
PX LH

60
PX FSH

KEL HIPOTHALAMUS

BILA

-30 LH -60 FSH

PITUITARY BAIK

GROUP I (BUAH DADA UTERUS )

PROD ESTROG NEGATIF

TINGGI

PX FSH SERUM

RENDAH

A. OVARIUM GAGAL HIPER - HIPOG

B. HIPOTAL-PITUITARI GAGAL HIPOG-HIPOG

PENATALAKSANAAN AMENOREA PRIMER GROUP 1 BUAH DADA -, UTERUS +, (SENTRAL/PERIFER)

TINGGI HYPER-HYPOG FSH

RENDAH/ NORMAL

TENSI

PERIFER (E OV. GAGAL

) TENSI N OV GGL
KARYOTYPING

SENTRAL (PITUITARI- HYPOTAL)

CT SCAN

TEST PROVOKASI ACTH

GGN. SINTESA E

TEST PROVOKASI

KEL +
ANDROGENIK

KEL -

GnRH CC

PROGESTERON 17 OH PROGEST SDKT NAIK

NON ANDROGENIK

HIPOTAL

GGN SINTESA E DEF. 17 HYDROGENASE

XO X/XX/XXX X/XXX
AWAS KEL ORGAN LAIN AUTOIMUN

ADA KOMP Y /& TANDA ANDROGEN

PITUITARI
PROLAKTI NOMA

GGN PERTBHN SEKS SEKUNDER ANDROGEN (TB)

FERTILITAS

CORTISON HRT
HRT

SP. PERTUMBHN OPTIMAL (3 BLN TUMBUH LAGI)

OOSIT DONOR IVF

GONADEKTOMI HRT

LAIN
BEDAH

BROMO KRIPTIN

COMPLETE DIAGNOSTIC EVALUTION OF WOMEN WITH GROUP I PRIMARY AMENORRHEA

LOW OR NORMAL

SERUM FSH LEVEL

ELEVATED HYPERGONADOTROPIC HYPOGONADISM

HYPOGONADOTROPIC HYPOGONADISM

CT OR MRI, PROLAKTIN TSH

BLOOD PRESSURE NORMAL BLOOD PRESSURE

HYPERTENSION

NO LESION

LESION

GYNADAL DYSGENESIS

GnRH STIMULATION TEST KARYOTYPING NORMAL FSH, LH RESPONSE HYPOTHALAMIC FAILURE ABSENT RESPONSE PITUITARI FAILURE 45,X 46,X/ABNORMAL X MOSAICISM PURE GONADAL, 45,X/ 46 XY 45,X/45,Xi (Yq)

17HYDROX DEFICIENCY 46, XX

45,X TESTICULAR DETERMINANT POSITION

MASHCHAK
GROUP BUAH DADA UTERUS
KETERANGAN
SENTRAL PERIFER
RKH TFS KEL SEKS KROMOSOM EVALUASI SPT AMENORE SEKUNDER

I
II III IV

GUP II AMENOREA
(BUAH DADA POS,UTERUS NEG)

ESTROG POS RKH GGAN PERTUMB ORGAN REPROD TFS

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 PERTUMB ORGAN REPROD TFS

RKH : MAYER ROKITANSKY KUSTER HAUSER SYNDROME

TFS : TESTISCULAR FEMINIZATION SYNDROME

NORMAL DEVELOPMENT OF INTERNAL AND EXTERNAL GENITAL

UNDIFFERENTIATED GONAD
KARYOTIPE

XX OVARIAN DEVELOPMENT

XY
TESTICULAR DEVELOPMENT

WOLFIAN DUCT REGRESION + MULLERIAN DEVELOPMENT OF OVIDUCTS AND UTERUS + FEMALE EXTERNAL GENITAL MIS

TESTOST. AND DEHYDROTEST

T
WOLFIAN OV. DEV.

DHT
DEV. OF MALE EXT GENITAL

MULLERIAN DUCT. REGRESSION

SEMINAL VES. AND PROSTAT

LAB SCROTAL FUSION+ PHALLIC DEV.

XY

NORMAL

TIDAK NORMAL (GGN PERTUMBUHAN)

TFS GENOTIPE: LAKI PENOTPE: WANITA

PERBEDAAN RKH DAN TFS

PEMERIKSAAN KARYOTIPING RKH 46 XX TFS 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 AND NORMAL PUBLIC AND AXILARY HAIR NORMAL MALE TESTOS LEVEL AND ABSENT PUBIC AND AXILARY HAIR

CONGENITAL ABSENT OF UTERUS

ANDROGEN INSENSITIVITY (TESTICULAR FEMINIZATION)

CONFIRM OVULATION WITH A BBT OR WEEKLY SERUM PROGESTERONE X 4

CONFIRM WITH KARYOTIPE

TERAPI AMENOREA GRUP II BUAH DADA (+) UTERUS (-)

RKH

TFS

BEDAH : VAGINA TIRUAN

GONADEKTOMI (PASCA PUBERTAS)

HRT

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

TESTOSTERON
TINGGI/ N, LAKI2 RENDAH/ N, WANITA

TANDA OVULASI KLINIS/ PMS BBT

RKH

USG

+
VAGINA TIRUAN
AWAS KEL. BAWAAN LAIN

TFS
GONADEKTOMI (PASCA PUBERTAS ) HRT

MASHCHAK
GROUP BUAH DADA UTERUS
KETERANGAN
SENTRAL PERIFER
RKH TFS KEL SEKS KROMOSOM EVALUASI SPT AMENORE SEKUNDER

I
II III IV

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 20.21 DESMOLASE 2 HYDROXYLASE PROGEST 11 HYDROXYLASE 18 HYDROXYLASE CORTICOST 18 OXYDASE . ALDOS

MINERALO CORTICOST

5 PRENENOLONE

DEOXY CORTICOST

18 hydroksi CORTICOST

17 HYDROXYLASE

GLUCOCORTICOST

517

HYDR PREGNOL

17 HYDROXY PROGESTERONE

DEOXY CORTISOL

CORTISOL

17,20 DESMOLASE

DEHYD EPIANDROS
5

ANDRO STENEDIONE
5

ESTRONE

SEX STEROID

17 OH STEROID OXYREDUCTASE
517

ANDRO STENEDIOL

TESTOSTERONE

ESTRADIOL

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