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+oral
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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.htm
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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.