48243123-Amenore-primer.ppt

7/22/2019 48243123-Amenore-primer.ppt

1/49

AMENOREAPRIMER

A.A.N ANANTASIKA


2/49


3/49


4/49

BATASANSPEROFF (2005)

I. GADIS USIA 14 TH TANDA SEKS SEKUNDER

(-) & BLM MENARKEII. GADIS USIA 16 TH TANDA SEKS SEKUNDER

POS TETAPI BELUM MENARKE


5/49

UMUR

(TH)

BUAH

DADA

HAID JENIS

14 PRIMER

16 (+) PRIMER

PASKA

MENARCHE

3 SIKLUS SEKUNDER

6 BULAN


6/49

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


7/49

MASHCHAKGROUP BUAH

DADA

UTERUS KETERANGAN

I SENTRALPERIFER

II RKHTFS

III KEL SEKSKROMOSOM

IV EVALUASI SPTAMENORE

SEKUNDER


8/49

PERTUMBUHAN BUAH DADAMERUPAKAN PETANDA

ADANYA

ESTROGEN


9/49

KLASIFIKASI SEKS SEKUNDER TANNER


10/49

TANNER STAGING

BREAST PUBIC HAIRSTAGE 1

(PREPUBERTAL)

ELEVATION OF PAPILA ONLY NO PUBIC HAIR

STAGE 2 ELEVATION OF BREAST ANDPAPILA AS SMALL MOUND,

AREOLA DIAMETER ENLARGEDMEDIAN AGE:9,8 YRS

SPARSE,LONG,PIGMENTED

HAIRLY CHIEFLY ALONG LABIA

MAJORA,MEDIAN AGE 10,5 YRS

STAGE 3 FURTHER ENLARGEMENT

WITHOUT SEPARATION OF

BREAST AND AREOLA

MEDIAN AGE 11,2 YRS

DARK,COARSE,CURLED HAIR

SPARSELY SPREAD OVERMONS

MEDIAN AGE 11,4 YRS

STAGE 4 SECONDARY MOUND OF AREOLAAND PAPILA ABOVE THE BREAST

MEDIAN AGE 12,1 YRS

ADULT TIPE HAIR,ABUNDANT

BUT LIMITED TO THE MONS

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


11/49

MASHCHAKGROUP BUAH

DADA

UTERUS KETERANGAN

I SENTRALPERIFER

II RKHTFS



SEKUNDER


12/49

GROUP I

(BUAH DADA UTERUS )PROD ESTROG

NEGATIF

PX FSH SERUMTINGGI RENDAH

A. OVARIUM GAGAL

HIPER - HIPOG

B. HIPOTAL-PITUITARIGAGAL

HIPOG-HIPOG


13/49

A. OVARIUM GAGAL

HIPER-HIPOG

E

KELAINAN ORGAN

(OVARIUM)

KELAINAN

SINTESA E (ENZYM)


14/49

A. OVARIUM GAGAL

HIPER-HIPOG

KEL KROMOSOM

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 (MURNIGONADAL DISGENESIS)

-46 XX DG DEF 17 HIDROK

SILASE

ANDROGENIK **

-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


15/49

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 OTITISMEDIA

-JANTUNG

-GINJAL

-TULI

-OSTEOPOROSIS

-RESIST INSULIN RINGAN

-HASHIMOTOS

THYROIDITIS

-ADDISONS DIS

-ALOPECIA

-VITILIGO


16/49

TERAPI SINDROMA TURNER

PERTUMBUHAN FERTILITAS

CONTINOUS DAILY LOW DOSE ANDROGEN

CESSATION OF LINEAR GROUP

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

PRESENT: OOCYTE

DONATION BY IVF-ET

FUTURE:CRYOPESERVATION

OF FUNCTIONAL OOCYTE

OBTAINED IN INFANCY


17/49

A. OVARIUM GAGAL

HIPER-HIPOG

E

KELAINAN ORGAN

(OVARIUM)

KELAINAN

SINTESA E (ENZYM)


18/49

17 HYDROXYLASE

AROMATASI

17,20 DESMOLASE

P450scc

STEROIDOGENESISCHOLESTEROL

PREGNENOLON

DHEA

17 OH

PREGNENOLONE

CORTICO

COST.

DEHYD.

TESTOST.

CORTISOL

D O C

TESTOSTERONE

DESOXY

CORTISOL

PROGEST.

ANDROST

17 OH PROGEST

ESTRONE ESTRADIOL

3

HYDROXYSTEROID

DEHYD

ROGENASE

17OH

STERO

ID

DEHYDROGENA

SE

11

HYDROXYLASE

ALDOST.

CORTISON

5REDUCTASE

21HYDROXYLASE

11 HSD

CORTISON

17 HYDROXYLASE


19/49


20/49

DEF.17 HIDROXYLASE

CORTISONE

ACTH


21/49

DEFISIENSI 17 HIDROXILASE

1. GENITALIA EKSTERNA WANITA

2. RETENSI NATRIUM DAN HIPOKALEMIA

3. HIPERTENSI


22/49

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/TETA


23/49

BLOOD PRESSURE MEASUREMENT IN DIAGNOSIS

GROUP I PRIMARY AMENORHEA

SERUM FSH LEVEL

ELEVATED

HIPERGONADOTROPIC

HIPOGANADISME

BLOOD PRESSURE MEASUREMENT

NORMAL BLOOD PRESSURE

GONADAL DISGENESIS

KARYOTIPE

HIPERTENSION

-17 HIDROX DEF

-46 XX

45,X46,X/ ABNORMAL X

MOSAICISME

PURE GONADAL DISGENESIS

45 X/ 46 XY

45 X/TESTICULER

DETERMINANT POSITION


24/49

GROUP I


NEGATIF


A. OVARIUM GAGAL

HIPER - HIPOG


HIPOG-HIPOG


25/49

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

HIPOTHALAMIC

FAILURE

ABSENT

RESPONSE

PITUITARY

FAILURE


26/49

TEST GnRH

100 G GnRH/HR IM1 MINGGU

DRH

BASAL

2X/15

MNT

100 G GnRH IV

BOLUS 30

3O 60

PX

LH

PX

FSH

KEL HIPOTHALAMUSBILA

-30 LH

-60 FSH PITUITARY BAIK


27/49

GROUP I


NEGATIF


A. OVARIUM GAGAL

HIPER - HIPOG


HIPOG-HIPOG

PENATALAKSANAAN AMENOREA PRIMER GROUP 1


28/49

PENATALAKSANAAN AMENOREA PRIMER GROUP 1

BUAH DADA -, UTERUS +, (SENTRAL/PERIFER)

FSHRENDAH/

NORMAL

TINGGI

HYPER-HYPOG

PERIFER (E )

OV. GAGAL

GGN. SINTESA E

TENSI

TEST PROVOKASI

ACTH

PROGESTERON

17 OH PROGEST SDKT

NAIK

GGN SINTESA E

DEF. 17 HYDROGENASE

CORTISON

HRT

TENSI N

OV GGL

KARYOTYPING

NON

ANDROGENIKANDROGENIK

XO

X/XX/XXX

X/XXX

ADA KOMP Y /&

TANDA ANDROGEN

GONADEKTOMI

HRT

AWAS

KEL ORGAN LAIN

AUTOIMUN

GGN PERTBHN

SEKS SEKUNDER FERTILITAS

ANDROGEN (TB)

SP. PERTUMBHNOPTIMAL (3 BLN TUMBUH

LAGI)HRT

OOSIT DONOR

IVF

SENTRAL

(PITUITARI- HYPOTAL)

CT SCAN

KEL + KEL -

HIPOTAL +

TEST PROVOKASI

GnRHCC

-

PITUITARI

PROLAKTI

NOMA

BROMO

KRIPTIN

BEDAH

LAIN

COMPLETE DIAGNOSTIC EVALUTION OF WOMEN WITH GROUP I PRIMARY AMENORRHEA


29/49

COMPLETE DIAGNOSTIC EVALUTION OF WOMEN WITH GROUP I PRIMARY AMENORRHEA

SERUM FSH

LEVELLOW OR NORMAL ELEVATED

HYPERGONADOTROPIC

HYPOGONADISM

CT OR MRI, PROLAKTIN

TSH

HYPOGONADOTROPIC

HYPOGONADISM

BLOOD PRESSURE

NO LESION

HYPERTENSIONNORMAL BLOODPRESSURE

LESION

17HYDROX

DEFICIENCY

46, XX

GYNADAL

DYSGENESIS

KARYOTYPING

GnRH STIMULATION TEST

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)

45,X

TESTICULAR

DETERMINANT

POSITION


30/49


31/49

MASHCHAKGROUP BUAH

DADA

UTERUS KETERANGAN

I SENTRALPERIFER

II RKHTFS



SEKUNDER


32/49

GUP II AMENOREA

(BUAH DADA POS,UTERUS NEG)

RKH TFS

ESTROG

POS

GGANPERTUMB

ORGAN

REPROD

RKH : MAYER ROKITANSKY KUSTER HAUSER SYNDROME

TFS : TESTISCULAR FEMINIZATION SYNDROME


33/49

KLASIFIKASI KELAINAN

PERTUMBUHAN DUKTUS MULLER

I. AGENESIS (RKH)

II. GANGGUAN FUSI VERTIKALA. OBSTRUKSI

B. NON OBSTRUKSI

III. GANGGUAN FUSI LATERAL

A. OBSTRUKSI

B. NON OBSTRUKSI


34/49

RKH

DASARNYA GENOTIPE WANITA

AGENESIS DUKTUS MULLER(OVARIUM NORMAL)

KLINIS

H P O BAIK OVULASI POSTESTOSTERON N WANITA

GANGGUAN PADA GENITALIA : VAGINA DAN UTERUS


35/49

GUP II AMENOREA

(BUAH DADA POS,UTERUS NEG)

RKH TFS

ESTROG

POS

GGANPERTUMB

ORGAN

REPROD

RKH : MAYER ROKITANSKY KUSTER HAUSER SYNDROME

TFS : TESTISCULAR FEMINIZATION SYNDROME

NORMAL DEVELOPMENT OF INTERNAL AND EXTERNAL GENITAL


36/49

UNDIFFERENTIATED GONAD

KARYOTIPE XYXX OVARIANDEVELOPMENT

TESTICULAR

DEVELOPMENT

MISTESTOST. AND DEHYDROTEST

WOLFIAN DUCT REGRESION

+

MULLERIAN DEVELOPMENT OF

OVIDUCTS AND UTERUS

+

FEMALE EXTERNAL GENITALT DHT

WOLFIAN OV.

DEV.

DEV. OF

MALE EXT

GENITAL

SEMINAL

VES. ANDPROSTAT

LAB SCROTAL

FUSION+PHALLIC DEV.

MULLERIAN DUCT.

REGRESSION


37/49

XY

NORMAL TIDAK NORMAL

(GGN PERTUMBUHAN)

TFS

GENOTIPE: LAKI

PENOTPE: WANITA


38/49

PERBEDAAN RKH DAN TFSPEMERIKSAAN 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


39/49

DIFFERENTATION OF THE CONGENITAL ABSENCE OF THE

UTERUS FROM THE ANDROGEN INSENSITIVITY SYNDROME

SERUM TESTOSTERONE &

OBSERVATION OF PUBIC ANDAXILARY HAIR

NORMAL MALE TESTOS

LEVEL AND ABSENT PUBIC

AND AXILARY HAIR

NORMAL FEMALE TESTOSTERON LEVEL

AND NORMAL PUBLIC

AND AXILARY HAIR

ANDROGEN INSENSITIVITY

(TESTICULAR FEMINIZATION)CONGENITAL ABSENT OF UTERUS

CONFIRM WITH KARYOTIPECONFIRM OVULATION WITH A BBT OR

WEEKLY SERUM PROGESTERONE X 4


40/49

TERAPI AMENOREA GRUP II

BUAH DADA (+) UTERUS (-)

RKH TFS

BEDAH :

VAGINA TIRUANGONADEKTOMI

(PASCA PUBERTAS)

HRT


41/49

PENATALAKSANAAN AMENOREA PRIMER GRUP II,

BUAH DADA +, UTERUS -, (RKH, TFS)

TESTOSTERON

TINGGI/ N,

LAKI2

RENDAH/

N, WANITA RKH

TANDA OVULASI

KLINIS/ PMS

BBT

USG

VAGINA TIRUAN

AWAS KEL. BAWAAN LAIN

TFS

-+

GONADEKTOMI

(PASCA PUBERTAS ) HRT


42/49

MASHCHAKGROUP BUAH

DADA

UTERUS KETERANGAN

I SENTRALPERIFER

II RKHTFS



SEKUNDER


43/49

EVALUATION OF WOMEN WITH GROUP III

PRIMARY AMENORRHEA

KARYOTIPE (46, XY)

ENZYME

DEFICIENCY AGONADISME


44/49

GROUP III AMENORE PRIMER

(BUAH DADA & UTERUS (-) )

A. GANGGUAN ENZIM

DEFISIENSI DARI:

- 17, 20 DESMOLASE *

- 17 - HIDROKSILASE *B. TESTICULAR AGONADISM

* KARYOTIPING XY GONADEKTOMI

CHOLESTEROL


45/49

CHOLESTEROL

5 ANDRO

STENEDIONE

17 HYDROXYPROGESTERONE

PROGEST

517 ANDROSTENEDIOL

517 HYDRPREGNOL

5 DEHYD

EPIANDROS

TESTOSTERONE

ESTRONE

DEOXY

CORTISOL

DEOXYCORTICOST

ESTRADIOL

CORTISOL

CORTICOST 18 hydroksiCORTICOST

5 PRENENOLONEALDOS

SE

XSTEROID

MINE

RALOCORTICOST

GLUCOC

ORTICOST

2 HYDROXYLASE3 HYDROXY DEHYDROGENASE

17 OH STEROIDOXYREDUCTASE

17,20 DESMOLASE

17 HYDROXYLASE

20.21 DESMOLASE18 OXYDASE .

18 HYDROXYLASE11 HYDROXYLASE


46/49

GROUP III AMENORE PRIMER

(BUAH DADA & UTERUS (-) )

A. GANGGUAN ENZIM

DEFISIENSI DARI:

- 17, 20 DESMOLASE *

- 17 - HIDROKSILASE *B. TESTICULAR AGONADISM

* KARYOTIPING XY GONADEKTOMI


47/49

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


48/49

THERAPI AMENORE PRIMER GRUP III

(BUAH DADA & UTERUS NEGATIF)

-GONADEKTOMI

-HRT


49/49

GRUP IV AMENOREA PRIMER

(BUAH DADA & UTERUS +)

EVALUASI

SAMA DENGAN AMENORE SEKUNDER

48243123-Amenore-primer.ppt

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