Gangguan Hormon Lain

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AHMAD FARIZ MALVI ZAMZAM ZEIN
SMF ILMU PENYAKIT DALAM RSUD WALED
Gangguan hormon
pertumbuhan
 What is Growth?
Growth is an increase in size of a tissue/organism
due to
- increase in cell size (hypertrophy)
- increase in number of cells (hyperplasia)
- increase in extracellular matrix around cells

hypertrophy

hyperplasia
HPA AXIS
Master Gland
 Growth Hormone

hormone
level

time
Normal Growth
Intrauterine life:
Prenatal (1.2-1.5 cm/w) mid gest (2.5 cm/w) bfore
birth (0.5 cm/w)
1st 2 y 15 cm/y
Middle childhood 6 cm/y
Pubertal growth 3-5 cm >
Adult maturity
Measurement
Height
Growth chart
Body proportions
Parental target height
Growth retardation
Primary growth abN
2nd growth disorder
Idiopathic short stature
Primary growth abN
Osteochondrodysplasia
Intrinsic abN of cartilage or bone
Feature: genetic transmission, abN in the size or shape of bones
(limbs, spine, skull), radiologic abN
Chromosomal abN
Downs syndrome (trisomy 21) ~ growth failure post natal &
associated w delayed skeletal maturation &pubertal growth spurt
Turners syndrome (gonadal dysgenesis) ~ short stature, delayed
puberty
2nd growth disorder
Malnutrition IGF insensitivity
GH deficiency
Hypothyroidism thyroxine ~ linear growth
Cushing syndrome:
GC >> GH secretion is reduced, inhibiting osteoblastic
activity, & enhances bone resorption
Tumor hipofisis
Tumbuh lambat, jinak, monoklonal
Sulit diobati & sering kambuh
10-15% neoplasma intrakranial
1-7/100.000 penduduk
Klasifikasi
Ukuran
Diameter < 1cm: mikroadenoma; > 1cm: makroadenoma
Pewarnaan histopatologi
Kromofobik vs kromofilik
Imunohistokimia
Functioning vs non functioning
Manifestasi klinis
< 1:
Defisiensi 1/lebih hormon
hipofisis
Kelebihan hormon
(terutama prolaktin, GH, &
ACTH)
Efek massa tumor
Ditemukan pada CT/MRI
Anamnesis
~ desakan massa
Penurunan libido, disfc ereksi,
amenorea, mudah lelah
PF
>> tampak sehat
Pemeriksaan Penunjang: Laboratorium
Evaluasi dasar hormonal Pengukuran dinamis kadar
hipofisis hormon
Prolaktin GH IGF1, ITT, GH-
RH/arginine, arginine
LH, FSH, testosteron
ACTH kortisol, LDCT, SDCT,
TSH, tiroksin ITT
ACTH, kortisol LH/FSH estradiol, FSH, LH,
IGF1 prolactin
TSH fT4, TSH
Pemeriksaan Penunjang: Radiologi
CT MRI
Cukup spesifik Gambaran yang lebih jelas
Dapat mendeteksi tumor struktur jaringan lunak &
dengan kalsifikasi vaskular
Lbh superior struktur tulan Kelemahan: mahal
dan kalsifikasi pada jaringan
lunak
Kelemahan (vs MRI): detail msh
inferior, pajanan radiasi >>
Tata laksana: Pembedahan
Transsphenoidal approach >>
Indikasi :
Umum: CSF leak, intoleransi thdp medikamentosa, perdarahan
pituitari, rekurensi, kompresi traktus visual/SSP
Khusus: acromegaly, nonfunctioning, Cushings disease,
prolactinoma, TSH-secreting adenoma
Tata laksana: Radioterapi
Indikasi :
Craniopharyngioma, hormone hypersecretion recurrence,
nonadenomatous invasive sellar mass, pituitary adenoma,
tumor reccurence
Tata laksana: Medikamentosa
Primer (terapi supresi hormon dengan bromokriptin &
analog somatostatin)
Terapi substitusi hormon (peri- & post operasi)
TERIMA KASIH