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7/27/2019 Moon Facies and Steroid Treatment
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Moon Facies and Steroid Treatment
Long-term use of steroids such asprednisonecan cause many of the same signs and symptoms of
Cushing's syndrome. Although more than 10 million Americans take these types of medications, many
may be affected by moon facies but not know its cause.
In fact, weight gain with fat redistribution such as moon facies is one of the most common signs of steroid
use. Your risk of developing these signs depends on the dose of medication and how long you take it.With steroid use, an increase in appetite and food intake may contribute to weight gain.
Symptoms usually occur as the result of long-term use of oral steroids. But less commonly, injected
orinhaled steroidsmay cause Cushing's signs and symptoms, too.
The best way to reduce the impact of symptoms is to reduce the dose of medication or discontinue it
altogether, but you should not do this on your own. If you need to continue using it, your doctor will have
you try the lowest effective dose. For example, taking the medication every other day can sometimes
lessen Cushingoid changes. If this does not resolve moon facies and other symptoms, your doctor may
suggest trying other types of therapies.
Cushing syndrome
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Cushing syndrome is a disorder that occurs when your body is exposed to high levels of the hormone
cortisol. It may also occur if you take too much cortisol or other steroid hormones.
See also:Cushing's disease (pituitary Cushing's)
Causes
Cushing syndrome may be caused by taking too muchcorticosteroid medications,such as prednisone
and prednisolone. These drugs are used to treat conditions such as asthmaandrheumatoid arthritis.
Other people develop Cushing syndrome because their bodies produce too much cortisol, a hormone
normally made in the adrenal gland. Causes of too much cortisol are:
Cushing's disease,when the pituitary gland makes too much of the hormoneACTH.ACTH then
signals the adrenal glands to produce cortisol. Tumor of the pituitary gland may cause this
condition.
Tumor of the adrenal gland
Tumor elsewhere in the body that produces cortisol
Tumors elsewhere in the body that produce ACTH (such as the pancreas, lung, and thyroid)
Symptoms
Most people with Cushing syndrome will have:
Upper body obesity (above the waist) and thin arms and legs
Round, red, full face (moon face)
Slow growth rate in children
Skin changes that are often seen:
http://www.webmd.com/drugs/mono-9383-PREDNISONE+-+ORAL.aspx?drugid=6007&drugname=prednisone+oralhttp://www.webmd.com/drugs/mono-9383-PREDNISONE+-+ORAL.aspx?drugid=6007&drugname=prednisone+oralhttp://www.webmd.com/drugs/mono-9383-PREDNISONE+-+ORAL.aspx?drugid=6007&drugname=prednisone+oralhttp://www.webmd.com/asthma/guide/asthma-control-with-anti-inflammatory-drugshttp://www.webmd.com/asthma/guide/asthma-control-with-anti-inflammatory-drugshttp://www.webmd.com/asthma/guide/asthma-control-with-anti-inflammatory-drugshttp://www.nlm.nih.gov/cgi/medlineplus/email_request.pl?refPage=http://www.nlm.nih.gov/medlineplus/ency/article/000410.htm&emailTitle=Cushing+syndromehttp://www.nlm.nih.gov/medlineplus/ency/article/000410.htmhttp://www.nlm.nih.gov/medlineplus/ency/article/000410.htmhttp://www.nlm.nih.gov/medlineplus/ency/article/000348.htmhttp://www.nlm.nih.gov/medlineplus/ency/article/000348.htmhttp://www.nlm.nih.gov/medlineplus/ency/article/000348.htmhttp://www.nlm.nih.gov/medlineplus/ency/article/000389.htmhttp://www.nlm.nih.gov/medlineplus/ency/article/000389.htmhttp://www.nlm.nih.gov/medlineplus/ency/article/000389.htmhttp://www.nlm.nih.gov/medlineplus/ency/article/000141.htmhttp://www.nlm.nih.gov/medlineplus/ency/article/000141.htmhttp://www.nlm.nih.gov/medlineplus/ency/article/000141.htmhttp://www.nlm.nih.gov/medlineplus/ency/article/000431.htmhttp://www.nlm.nih.gov/medlineplus/ency/article/000431.htmhttp://www.nlm.nih.gov/medlineplus/ency/article/000431.htmhttp://www.nlm.nih.gov/medlineplus/ency/article/000348.htmhttp://www.nlm.nih.gov/medlineplus/ency/article/000348.htmhttp://www.nlm.nih.gov/medlineplus/ency/article/003695.htmhttp://www.nlm.nih.gov/medlineplus/ency/article/003695.htmhttp://www.nlm.nih.gov/medlineplus/ency/article/003695.htmhttp://www.nlm.nih.gov/medlineplus/ency/article/000407.htmhttp://www.nlm.nih.gov/medlineplus/ency/article/000407.htmhttp://www.nlm.nih.gov/medlineplus/ency/article/003105.htmhttp://www.nlm.nih.gov/medlineplus/ency/article/003105.htmhttp://www.nlm.nih.gov/medlineplus/ency/article/003105.htmhttp://www.nlm.nih.gov/medlineplus/ency/article/003105.htmhttp://www.nlm.nih.gov/medlineplus/ency/article/000407.htmhttp://www.nlm.nih.gov/medlineplus/ency/article/003695.htmhttp://www.nlm.nih.gov/medlineplus/ency/article/000348.htmhttp://www.nlm.nih.gov/medlineplus/ency/article/000431.htmhttp://www.nlm.nih.gov/medlineplus/ency/article/000141.htmhttp://www.nlm.nih.gov/medlineplus/ency/article/000389.htmhttp://www.nlm.nih.gov/medlineplus/ency/article/000348.htmhttp://www.nlm.nih.gov/medlineplus/ency/article/000410.htmhttp://www.nlm.nih.gov/medlineplus/ency/article/000410.htmhttp://www.nlm.nih.gov/medlineplus/ency/article/000410.htmhttp://www.nlm.nih.gov/cgi/medlineplus/email_request.pl?refPage=http://www.nlm.nih.gov/medlineplus/ency/article/000410.htm&emailTitle=Cushing+syndromehttp://www.nlm.nih.gov/cgi/medlineplus/email_request.pl?refPage=http://www.nlm.nih.gov/medlineplus/ency/article/000410.htm&emailTitle=Cushing+syndromehttp://www.webmd.com/asthma/guide/asthma-control-with-anti-inflammatory-drugshttp://www.webmd.com/drugs/mono-9383-PREDNISONE+-+ORAL.aspx?drugid=6007&drugname=prednisone+oral7/27/2019 Moon Facies and Steroid Treatment
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Acneor skin infections
Purple marks (1/2 inch or more wide) called striae on the skin of the abdomen, thighs, and
breasts
Thin skin with easy bruising
Muscle and bone changes include:
Backache, which occurs with routine activities
Bone pain or tenderness
Collection of fat between the shoulders (buffalo hump)
Rib and spine fractures (caused by thinning of the bones)
Weak muscles
Women with Cushing syndrome often have:
Excess hair growth on the face, neck, chest, abdomen, and thighs
Menstrual cycle that becomes irregular or stops
Men may have:
Decreased or no desire for sex
Impotence
Other symptoms that may occur with this disease:
Mental changes,such as depression, anxiety, or changes in behavior
Fatigue
Headache
Increased thirst and urination
Exams and Tests
Blood sugarandwhite blood cell countsmay be high.Potassium levelmay be low.
Laboratory tests that may be done to diagnose Cushing syndrome and identify the cause are:
Serumcortisol levels
Salivary cortisol levels
Dexamethasone suppression test 24-hour urine forcortisolandcreatinine
ACTH level
ACTH (cosyntropin) stimulation test
Tests to determine the cause or complications may include:
Abdominal CT
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ACTH test
PituitaryMRI
Bone density,as measured by dual x-ray absorptiometry (DEXA)
High cholesterol,including high triglycerides and low high-density lipoprotein (HDL) may also be present.
Treatment
Treatment depends on the cause.
Cushing syndrome caused by corticosteroid use:
Slowly decrease the drug dose (if possible) under medical supervision.
If you cannot stop taking the medication because of disease, your high blood sugar, high
cholesterol levels, and bone thinning orosteoporosisshould be closely monitored.
Cushing syndrome caused by a pituitary or a tumor that releases ACTH (Cushing's disease):
Surgery to remove the tumor
Radiation after removal of a pituitary tumor (in some cases)
You may need hydrocortisone (cortisol) replacement therapy after surgery, and possibly
continued throughout your life
Cushing syndrome due to anadrenal tumororother tumors:
Surgery to remove the tumor
If the tumor cannot be removed, medications to help block the release of cortisol
Outlook (Prognosis)
Removing the tumor may lead to full recovery, but there is a chance that the condition will return.
Survival for people with ectopic tumors depends on the tumor type. Untreated, Cushing syndrome can be
life-threatening.
Possible Complications
Diabetes
Enlargement of pituitary tumor
Fracturesdue to osteoporosis
High blood pressure
Kidney stones
Serious infections
When to Contact a Medical Professional
Call your health care provider if you have symptoms of Cushing syndrome.
Alternative Names
http://www.nlm.nih.gov/medlineplus/ency/article/003335.htmhttp://www.nlm.nih.gov/medlineplus/ency/article/003335.htmhttp://www.nlm.nih.gov/medlineplus/ency/article/003335.htmhttp://www.nlm.nih.gov/medlineplus/ency/article/007197.htmhttp://www.nlm.nih.gov/medlineplus/ency/article/007197.htmhttp://www.nlm.nih.gov/medlineplus/ency/article/000403.htmhttp://www.nlm.nih.gov/medlineplus/ency/article/000403.htmhttp://www.nlm.nih.gov/medlineplus/ency/article/000389.htmhttp://www.nlm.nih.gov/medlineplus/ency/article/000389.htmhttp://www.nlm.nih.gov/medlineplus/ency/article/000360.htmhttp://www.nlm.nih.gov/medlineplus/ency/article/000360.htmhttp://www.nlm.nih.gov/medlineplus/ency/article/000360.htmhttp://www.nlm.nih.gov/medlineplus/ency/article/000407.htmhttp://www.nlm.nih.gov/medlineplus/ency/article/000407.htmhttp://www.nlm.nih.gov/medlineplus/ency/article/000407.htmhttp://www.nlm.nih.gov/medlineplus/ency/article/000406.htmhttp://www.nlm.nih.gov/medlineplus/ency/article/000406.htmhttp://www.nlm.nih.gov/medlineplus/ency/article/000406.htmhttp://www.nlm.nih.gov/medlineplus/ency/article/001214.htmhttp://www.nlm.nih.gov/medlineplus/ency/article/001214.htmhttp://www.nlm.nih.gov/medlineplus/ency/article/000001.htmhttp://www.nlm.nih.gov/medlineplus/ency/article/000001.htmhttp://www.nlm.nih.gov/medlineplus/ency/article/000458.htmhttp://www.nlm.nih.gov/medlineplus/ency/article/000458.htmhttp://www.nlm.nih.gov/medlineplus/ency/article/000458.htmhttp://www.nlm.nih.gov/medlineplus/ency/article/000001.htmhttp://www.nlm.nih.gov/medlineplus/ency/article/001214.htmhttp://www.nlm.nih.gov/medlineplus/ency/article/000406.htmhttp://www.nlm.nih.gov/medlineplus/ency/article/000407.htmhttp://www.nlm.nih.gov/medlineplus/ency/article/000360.htmhttp://www.nlm.nih.gov/medlineplus/ency/article/000389.htmhttp://www.nlm.nih.gov/medlineplus/ency/article/000403.htmhttp://www.nlm.nih.gov/medlineplus/ency/article/007197.htmhttp://www.nlm.nih.gov/medlineplus/ency/article/003335.htm7/27/2019 Moon Facies and Steroid Treatment
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Hypercortisolism
References
Stewart PM, Krone NP. The adrenal cortex. In: Kronenberg HM, Shlomo M, Polonsky KS, Larsen PR,
eds.Williams Textbook of Endocrinology. 12th ed. Philadelphia, Pa: Saunders Elsevier;2011:chap 15.
Update Date: 12/11/2011
Updated by: Nancy J. Rennart, MD, Chief of Endocrinology & Diabetes, Norwalk Hospital, Associate
Clinical Professor of Medicine, Yale University School of Medicine. Review provided by VeriMed
Healthcare Network. Also reviewed by David Zieve, MD, MHA, Medical Director, A.D.A.M., Inc.
Browse the Encyclopedia
Cushing's Sindrome
A. Defenisi
Kortisol plasma berlebihan menyebabkan suatu keadaan yang disebut dengan cushing
syndrome, dimana aldosteron berlebihan menyebabkanaldosteronisme, dan androgen adrenal
berlebihan menyebabkan virilismeadrenal. Sindrom ini tidak dijumpai dalam bentuk murni tetapi
bisa mempunyai gambaran yang tumpang tindih.
A. Defenisi
Kortisol plasma berlebihan menyebabkan suatu keadaan yang disebut dengan cushing
syndrome, dimana aldosteron berlebihan menyebabkanaldosteronisme, dan androgen adrenal
berlebihan menyebabkan virilismeadrenal. Sindrom ini tidak dijumpai dalam bentuk murni tetapibisa mempunyai gambaran yang tumpang tindih.
B. Etiologi dan Klasifikasi
Cushing melukiskan suatu sindrom yang ditandai dengan obesitas badan (truncul
obesity), hipertensi, mudah lelah kelemahan, amenorea, hirsutisme, striae abdomen berwarna
ungu, edema, glukosuria, osteoporosis, dan tumor basofilik hipofisis. Sindrom ini dinamakan
dengan sindrom cushing . Adapun klasifikasinya adalah sebagai berikut :
1. Hiperplasia Adrenal
a. Sekunder terhadap kelebihan produksi ACTH hipofisis, yaitu berupa disfungsi hipothalamik-
hipofisa dan mikro dan makroadenoma yang menghasilkan ACTH hipofisis.
b. Sekunder terhadap Tumor non endokrin yang menghasilkan ACTH atau CRH, yaitu karsinoma
Bronkhogenik, karsinoid Thymus, karsinoma pankreas, dan adenoma bronkhus.
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2. Hiperplasia noduler adrenal, yaitu neoplasia adrenal berupa adenoma dan karsinoma
3. Penyebab eksogen atau iatrogenik yang disebabkan penggunaan glukokortikoid jangka lama
penggunaan ACTH jangka lama
Tanpa mempertimbangkan etiologi semua kasus cushing sindrom endogen disebabkanoleh peningkatan sekresi hormon kortisol oleh adrenal. Pada kebanyakan kasus penyebabnya
ialah :
1. Hiperplasia adrenal bilateral oleh karena hipersekresi ACTH hipofisis
2. Produksi ACTH oleh tumor non-endokrin
3. 20-25% pasien sindrom Cushing menderita neoplasma adrenal
4. Penyebab terbanyak adalah iatrogenik
C. Epidemiologi
Pada sindroma Cushing berupa sindroma ektopik ACTH lebih sering pada laki-laki
dengan rasio 3:1, pada insiden hiperplasia hipofisis adrenal adalah lebih besar pada wanita
daripada laki-laki, kebanyakan muncul pada usia dekade ketiga atau keempat.
D. Patofisiologi
Penyebab terjadinya hipersekresi ACTH hipofisis masih diperdebatkan. Beberapa peneliti
berpendapat bahwa defek adalah adenoma hipofisis, pada beberapa laporan dijumpai tumor-tumor pada lebih 90% pasien dengan hiperplasia adrenal tergantung hipofisis. Disamping itu,
defek bisa berada pada hipothalamus atau pada pusat-pusat saraf yang lebih tinggi, menyebabkan
pelepasan CRH (Corticotropin Relasing Hormone) yang tidak sesuai dengan keadaan kortisol
yang beredar. Konsekuensinya akan membutuhkan kadar kortisol yang lebih tinggi untuk
menekan sekresi ACTH ke rentang normal. Defek primer ini menyebabkan hiperstimulasi
hipofisis, menyebabkan hiperplasia atau pembentukan tumor. Pada waktu ini tumor hipofisis
menjadi independen dari pengaruh pengaturan sistem saraf pusat dan/atau kadar kortisol yang
beredar. Pada serangkaian pembedahan, kebanyakan individu yang hipersekresi ACTH hipofisis
menderita adenoma (diameter 10mm) atau hiperplasia difusa sel-sel kortikotropik.
Tumor nonendokrin bisa mensekresi polipeptida yang secara biologik, kimiawi, dan
immunologik takk dapat dibedakan dari ACTH dan CRHdan menyebabkan hiperplasia bilateral.
Kebanyakan dari kasus ini berkaitan denganprimitive small cell(Oat Cell) tipe dari karsinoma
bronkogenik atau tumor timus, pankreas, ovarium, Ca. Medulla tiroid, atau adenoma Bronkus.
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Timbulnya sindrom Cushing bisa mendadak, terutama pada pasien dengan Ca. Paru, pasien tidak
memperilahtkan gambaran klinis. Sebaliknya pasien dengan tumor karsinoid atau
feokromositoma mempunyai perjalanan klinis yang lama dan menunjukkan gambaran
Cushingoid yang tipikal Hiperpigmentasi pada penderita sindrom Cushing hampir selalu
menunjukkan tumor ekstra adrenal, di luar kranium atau dalam kranium.
Tumor atau neoplasma adrenal unilateral dan kira-kira setengahnya adalah ganas
(maligna). Pasien kadang-kadang mempunyai gambaran biokimia hipersekresi ACTH hipofisis,
individu ini biasanya mempunyai mikro atau makronudular kedua kelenjar nodular
mengakibatkan hiperplasi nodular. Penyebabnya adalah penyakit autoimun familial pada anak-
anak atau dewasa muda (disebut displasia korteks multinodular berpigmen) dan hipersensitivitas
terhadapgastric inhibitory polypeptide, mungkin sekunder terhadap peningkatan ekspresi
reseptor untuk peptida di korteks adrenal. Penyebab terbanyak sindrom Cushing adalah
iatrogenik pemberian steroid eksogen dengan berbagai alasan.
E. Gejala klinis
Mobilisasi jaringan ikat suportif perifer menyebabkan kelemaha otot dan kelelahanm
osteoporosis, striae kulit, dan mudah berdarah di bawah kulit. Peningkatan glukoneogenesis hati
dan resistensi insulin dapat menyebabkan gangguan toleransi glukosa. Hiperkortisolisme
mendorong penumpukan jaringan adiposa di tempat-tempat tertentu khususnya wajah bagian atas
(Moon face), daerah antara tulang belikat (Bufallo Hump) dan mesentrik (Obesitas Badan).
Jarang, tumor episternal dan pelebaran mediastinum sekunder terhadap penumpukan lemak.
Alasan untuk distribusi yang aneh dari jaringan adiposa ini belum diketahui, tetapi berhubungan
dengan resistensi insulin dan/atau peningkatan kadar insulin. Wajah tampak pletorik, tanpa
disertai peningkatan sel darah merah. Hipertensi sering terjadi dan bisa dijumopai perubahan
emosional, mudah tersinggung, emosi labil, depresi berat, bingung atau psikosis. Pada wanita
peningkatan kadar androgen adrenal menyebabkan acne, hirsutisme, dan oligomenorrea atau
amenorrea, simtom yang lain seperti obesitas, hipertensi, osteoporosis, dan DM kurang
membantu diagnosis. Sebaliknya tanda-tanda mudah berdarah, striae, miopati, dan virilisasi
adalah lebih sugestif pada sindrom Cushing. Kecuali pada sindrom Cushing iatrogenik, kadar
kortisol plasma dan urin meningkat. Kadang-kadang hipokalemia, dan alkalosis metabolik
dijumpai, terutama dengan produksi ACTH ektopik.
F. Diagnosis
Diagnosis sindrom Cushing bergantung pada kadar produksi kortisol dan kegagalan
menekan produksi kortisol secara normal bila diberikan deksametason.
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Untuk skrining awal dilakukan ters supresi deksametason tengah malam. Pada kasus sulit
(Obesitas), pengukuran kortisol bebas urin 24 jam juga bisa digunakan sebagai tes skrining awal.
Bila kadar kortisol bebas urin lebih tinggi dari 275 nmol/dl (100 mikrogram/dL), diagnosis
defenitif ditetapkan bila gagal menurunkan kortisol urin menuju ke
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memproduksi glukokortikoid, zona glomerulosa bisa terganggu. Obat ini biasanya diberikan
dengan dosis terbagi tiga sampai empat kali sehari, dengan dosis ditingkatkan secara bertahap
menjadi 8 sampai 10g perhari. Semua pasien yang diobati dengan mitotan harus menjalani terapi
pemulihan jangka lama.
b. Hiperplasia Bilateral
Terapi yang harus ditujukan untuk mengurangi kadar ACTH, pengobatan ideal adalah
pengangkatan dengan menjalani eksplorasi bedah hipofisis via trans-sfenoidal dengan harapan
menemukan adenoma. Pada banyak keadaan dianjurkan selective petrosal sinus venous sampling
dan adrenalektomi total. Penghambatan steroidogenesis juga bisa diindikasikan pada subjek
cushingoid berat sebelum intervensi pembedahan. Adrenalektomi kimiawi mungkin lebih unggul
dengan pemberian penghambat steroidogenesis ketokonazol (600-1200mg/hari). Mitotan (2-
3mg/hari) dan/atau penghambatan sintesis sterooid aminoglutetimid (1g/hari) dan metiraponi (2-
3g/hari). Mifeperistone, suatu inhibitor kompetitif ikatan glukokortikoid terhadap reseptornya,bisa menjadi pilihan pengobatan.
H. Prognosis
Adenoma adrenal yang berhasil diobati dengan pembedahan mempunyai prognosis baik
dan tidak mungkin kekambuhan terjadi. Prognosis tergantung pada efek jangka lama dari
kelebihan kortisol sebelum pengobatan, terutama aterosklerosis dan osteoporosis. Prognosis
karsinoma Adrenal adalah amat jelek, disamping pembedahan.
DAFTAR PUSTAKA
Arif Mansjoer, Suphohaita dan Wahyu Ika Wardhani, 2000,Kapita Selekta Kedokteran, Edisi Ketiga
Jilid Kedua
Guyton & Hall. 2006.Medical Physiology Eleventh Edition. Philadelphia: Elsevier Saunders
Price, Sylvia A, Wilson, Lorraine M. 2006.Patofisiologi 2. Jakarta : EGC.
Robbins, Cotran, Kumar. 1996. Dasar Patologi Penyakit. Jakarta: EGC.
Sudoyo, Aru W, dkk (editor). 2006.Ilmu Penyakit Dalam Jilid III Edisi IV. Jakarta : Departemen Ilmu
Penyakit Dalam FK UI.
How is Cushing's syndrome diagnosed?
Diagnosis is based on a review of the patient's medical history, physical examination and laboratory
tests. Often x-ray exams of the adrenal or pituitary glands are useful for locating tumors. These tests
help to determine if excess levels of cortisol are present and why.
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24-Hour Urinary Free Cortisol Level
This is the most specific diagnostic test. The patient's urine is collected over a 24-hour period and
tested for the amount of cortisol. Levels higher than 50-100 micrograms a day for an adult suggest
Cushing's syndrome. The normal upper limit varies in different laboratories, depending on which
measurement technique is used.
Once Cushing's syndrome has been diagnosed, other tests are used to find the exact location of the
abnormality that leads to excess cortisol production. The choice of test depends, in part, on the
preference of the endocrinologist or the center where the test is performed.
Dexamethasone Suppression Test
This test helps to distinguish patients with excess production of ACTH due to pituitary adenomas
from those with ectopic ACTH-producing tumors. Patients are givendexamethasone,a synthetic
glucocorticoid, by mouth every 6 hours for 4 days. For the first 2 days, low doses of dexamethasone
are given, and for the last 2 days, higher doses are given. Twenty-four hour urine collections are
made before dexamethasone is administered and on each day of the test. Since cortisol and other
glucocorticoids signal the pituitary to lower secretion of ACTH, the normal response after taking
dexamethasone is a drop in blood and urine cortisol levels. Different responses of cortisol to
dexamethasone are obtained depending on whether the cause of Cushing's syndrome is a pituitary
adenoma or an ectopic ACTH-producing tumor.
The dexamethasone suppression test can produce false-positive results in patients with depression,
alcohol abuse, high estrogen levels, acute illness, and stress. Conversely, drugs such
asphenytoinand phenobarbital may cause false-negative results in response to dexamethasone
suppression. For this reason, patients are usually advised by their physicians to stop taking these
drugs at least one week before the test.
CRH Stimulation Test
This test helps to distinguish between patients with pituitary adenomas and those with ectopic ACTH
syndrome or cortisol-secreting adrenal tumors. Patients are given an injection of CRH,
thecorticotropin-releasing hormonewhich causes the pituitary to secrete ACTH. Patients with
pituitary adenomas usually experience a rise in blood levels of ACTH and cortisol. This response is
rarely seen in patients with ectopic ACTH syndrome and practically never in patients with cortisol-
secreting adrenal tumors.
Direct Visualization of the Endocrine Glands (Radiologic Imaging)
Imaging tests reveal the size and shape of the pituitary and adrenal glands and help determine if a
tumor is present. The most common are the CT (computerized tomography) scan and MRI
(magnetic resonance imaging). ACT scanproduces a series of x-ray pictures giving a cross-
sectional image of a body part. MRI also produces images of the internal organs of the body but
without exposing the patient to ionizing radiation.
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Imaging procedures are used to find a tumor after a diagnosis has been established. Imaging is not
used to make the diagnosis of Cushing's syndrome because benign tumors, sometimes called
"incidentalomas," are commonly found in the pituitary and adrenal glands. These tumors do not
produce hormones detrimental to health and are not removed unless blood tests show they are a
cause of symptoms or they are unusually large. Conversely, pituitary tumors are not detected by
imaging in almost 50 percent of patients who ultimately require pituitary surgery for Cushing's
syndrome.
Petrosal Sinus Sampling
This test is not always required, but in many cases, it is the best way to separate pituitary from
ectopic causes of Cushing's syndrome. Samples of blood are drawn from the petrosal sinuses, veins
which drain the pituitary, by introducing catheters through a vein in the upper thigh/groin region, with
local anesthesia and mild sedation. X-rays are used to confirm the correct position of the catheters.
Often CRH, the hormone which causes the pituitary to secrete ACTH, is given during this test to
improve diagnostic accuracy. Levels of ACTH in the petrosal sinuses are measured and compared
with ACTH levels in a forearm vein. ACTH levels higher in the petrosal sinuses than in the forearm
vein indicate the presence of a pituitary adenoma; similar levels suggest ectopic ACTH syndrome.
The Dexamethasone-CRH Test
Some individuals have high cortisol levels, but do not develop the progressive effects of Cushing's
syndrome, such as muscleweakness,fractures and thinning of the skin. These individuals may have
Pseudo Cushing's syndrome, which was originally described in people who were depressed or drank
excess alcohol, but is now known to be more common. Pseudo Cushing's does not have the same
long-term effects on health as Cushing's syndrome and does not require treatment directed at the
endocrine glands. Although observation over months to years will distinguish Pseudo Cushing's from
Cushing's, the dexamethasone-CRH test was developed to distinguish between the conditions
rapidly, so that Cushing's patients can receive prompt treatment. This test combines the
dexamethasone suppression and the CRH stimulation tests. Elevations of cortisol during this test
suggest Cushing's syndrome.
Some patients may have sustained high cortisol levels without the effects of Cushing's syndrome.
These high cortisol levels may be compensating for the body's resistance to cortisol's effects. This
rare syndrome of cortisol resistance is a genetic condition that causes hypertensionand chronic
androgen excess.
Sometimes other conditions may be associated with many of the symptoms of Cushing's syndrome.
These includepolycystic ovarian syndrome,which may cause menstrual disturbances,weight
gainfrom adolescence, excess hair growth and sometimes impaired insulin action anddiabetes.
Commonly, weight gain, high blood pressure and abnormal levels ofcholesteroland triglycerides in
the blood are associated with resistance to insulin action and diabetes; this has been described as
the "Metabolic Syndrome-X." Patients with these disorders do not have abnormally elevated cortisol
levels.
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Pemeriksaan Penunjang Cushing Syndrome
1. Pada pemeriksaan laboratorium sederhana, didapati limfositofeni, jumlah netrofil antara 10.000
25.000/mm3. eosinofil 50/ mm3 hiperglekemi (Dm pada 10 % kasus) dan hipokalemia.
2. Pemeriksaan laboratorik diagnostik. Pemeriksaan kadar kortisol dan overnight dexamethasonesuppression test yaitu memberikan 1 mg dexametason pada jam 11 malam, esok harinya diperiksa lagi
kadar kortisol plasma. Pada keadaan normal kadar ini menurun. Pemerikaan 17 hidroksi kortikosteroid
dalam urin 24 jam (hasil metabolisme kortisol), 17 ketosteroid dalam urin 24 jam.
3. Tes-tes khusus untuk membedakan hiperplasi-adenoma atau karsinoma :
a. Urinary deksametasone suppression test. Ukur kadar 17 hidroxikostikosteroid dalam urin 24 jam,
kemudian diberikan dexametasone 4 X 0,5 mg selama 2 hari, periksa lagi kadar 17 hidroxi kortikosteroid
bila tidak ada atau hanya sedikit menurun, mungkin ada kelainan. Berikan dexametasone 4 x 2 mg
selama 2 hari, bila kadar 17 hidroxi kortikosteroid menurun berarti ada supresi-kelainan adrenal itu
berupa hiperplasi, bila tidak ada supresi kemungkinan adenoma atau karsinoma.
b. Short oral metyrapone test. Metirapone menghambat pembentukan kortisol sampai pada 17
hidroxikortikosteroid. Pada hiperplasi, kadar 17 hidroxi kortikosteroid akan naik sampai 2 kali, pada
adenoma dan karsinoma tidak terjadi kenaikan kadar 17 hidroxikortikosteroid dalam urine.
c. Pengukuran kadar ACTH plasma.
d. Test stimulasi ACTH, pada adenoma didapati kenaikan kadar sampai 23 kali, pada kasinoma tidak
ada kenaikan (Mansjoer, 2007)(buku panduan asuhan keperawatan)
PenatalaksanaanCushing Syndrome
Pengobatan sindrom cushing tergantung ACTH tidak seragam, bergantung apakah sumber ACTH adalah
hipofisis / ektopik.
a. Jika dijumpai tumor hipofisis. Sebaiknya diusahakan reseksi tumor tranfenoida.
b. Jika terdapat bukti hiperfungsi hipofisis namun tumor tidak dapat ditemukan maka sebagai gantinya
dapat dilakukan radiasi kobait pada kelenjar hipofisis.
c. Kelebihan kortisol juga dapat ditanggulangi dengan adrenolektomi total dan diikuti pemberian kortisol
dosis fisiologik.
d. Bila kelebihan kortisol disebabkan oleh neoplasma disusul kemoterapi pada penderita dengan
karsinoma/ terapi pembedahan.
e. Digunakan obat dengan jenis metyropone, amino gluthemide yang bisa mensekresikan kortisol (Silvia
A. Price ; Patofisiologi Edisi 4 hal 1093).
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What is an MRI scan?
An MRI (or magnetic resonance imaging) scan is a radiology technique that uses magnetism, radio
waves, and a computer to produce images of body structures. The MRI scanner is a tube
surrounded by a giant circular magnet. The patient is placed on a moveable bed that is inserted into
the magnet. The magnet creates a strong magnetic field that aligns the protons of hydrogen atoms,which are then exposed to a beam of radio waves. This spins the various protons of the body, and
they produce a faint signal that is detected by the receiver portion of the MRI scanner. The receiver
information is processed by a computer, and an image is produced.
The image and resolution produced by MRI is quite detailed and can detect tiny changes of
structures within the body. For some procedures, contrast agents, such as gadolinium, are used to
increase the accuracy of the images.