ADRENAL
- Cushing’s Syndrome -
OLEH :
Woro Harjaningsih
Definisi
A. Cushing’s disease :
Dapat tjd :
• secara spontan cushing’s syndrome
spontaneous atau , merupakan penyebab
terbesar, kejadian kira2 2 – 4 kasus / juta populasi
dan pd wanita 9 X lebih banyak drpd laki-laki
• sebagai hasil pemberian kronik (jangka panjang)
kortikosteroid Cushing’s syndrome iatrogenic
Spontaneous cushing’s syndrome / Cushing’s disease
bisa tjd akibat :
• Adenoma hipofisis sekresi berlebih ACTH (> 90%)
• Hiperplasia difuse / pituitary corticotroph cells yg
bertanggung jawab thdp hipersekresi ACTH
Hiperplasia kmk krn hipersekresi CRH oleh hipotalamus
atau tumor pensekresi CRH (CRH-secreting tumors)
Hipersekresi kronik CRH tidak menyebabkan adenoma
hipofisis
B. Ectopic ACTH Syndrome
Tumor non hipofisis mensintesis & mensekresikan secara berlebih
(hipersekresi) ACTH yg aktif secara biologi atau peptida menyerupai
ACTH (ACTH-like peptide)
Biasanya karsinoma sel kecil dari paru atau tumor karsinoid dari
bronkhial
Lebih banyak menyerang laki-laki
C. ECTOPIC CRH Syndrome
• Sangat jarang menyebabkan Cushing’s Syndrome
• Sebagian besar kasus berhub dg tumor karsinoid bronkhial
D. Functioning Adrenocortical Tumors
Baik adrenocortical adenoma dan carcinoma menyebabkan Cushing’s
syndrome melalui pengeluaran kortisol secara autonom
Adenoma memp vaskuler yang tinggi, dengan area nekrosis,
perdarahan, degenerasi kista dan kalsifikasi bisa metastase ke ginjal,
retroperitoneum, liver dan paru
E. Adrenal Micronodular Hyperplasia
Jarang menyebabkan Cushing’s Syndrome
Sekitar separuh kasus timbul secara mendadak pada anak2 dan dewasa
muda
F. Adrenal Macronodular Hyperplasia
Jarang menyebabkan Cushing’s Syndrome
Beberapa pasien dg macronodular hyperplasia tidak menunjukkan
gambaran cushingoid tipikal
Patofisiologi
Patofisiologi
ACTH dependent
Cushing’s disease
Ectopic ACTH syndrome
Ectopic corticotropin-releasing hormone syndrome*
ACTH independent
Iatrogenic
Adrenal adenoma
Micronodular hyperplasia*
Macronodular hyperplasia*
Plasma
Kortisol 20 - 90
(μg/24 h)
Sasaran terapi
Dosing
Etiology Non drug Drug Initial Usual Max
Ectopic Surgery Metyrapone 1 – 1, 5 g/d, 1 – 6 g/d, 6 g/d
ACTH syndrome Chemotherapy tabs divided q4-6 h divided q4-6
Irradiation 250 mg h
Aminoglutethi 0,5-1 g/d, 1 g/d, 2 g/d
mide tabs, 250 divided 96 bid – divided q6
mg qid x 2 weeks
Pituitary Surgery Cyproheptadin 4 mg bid 24 – 32 32 mg/d
dependent Irradiation e, 2 mg/5 mL mg/d.,
syrup or 4 mg divided qid
tabs
Mitotane tabs
500 mg 1-6 g/d, 16 g/d
increased by 1-2 9 – 10 g/d,
g/d q3-7d divided tid –
Metyrapone See above qid
See above See above
• Steroid inhibitor
• Adrenolitik agent
mitotan
• Neuromodulator agent
• Antagonis reseptor
glukokortikoid
Steroid Inhibitor
• Cyproheptadine
• Bromocriptine
• Valproic acid
• Octreotide
Cyproheptadine
• 4Be aware that surgery on the pituitary may cause ACTH levels to fall
below normal, which is typical. Therefore, it may become necessary to
follow up with administration of a synthetic form of cortisol, such as
hydrocortisone or prednisone.
• 5Expect that radiotherapy or a combination of radiation therapy and
medication may be necessary if you're not a good candidate for surgery.
While radiation works to shrink tumors, drugs such as mitotane
(Lysodren) help to inhibit cortisol production.
• 6Know that there are several medications used to control cortisone
secretion, including mitotane, aminoglutethimide, metyrapone,
trilostane and ketoconazole.
•
Read more: How to Treat Cushing's Syndrome | eHow.com
http://www.ehow.com/how_2048201_treat-cushings-
syndrome.html#ixzz12pFKoNXq
Monitoring terapi