Archive | March 2017

Part III Cushing’s Syndrome – Diagnosing it and Treatment for it.


 

How is Cushing’s syndrome diagnosed and treated?

Diagnosis is established on a survey of the patient’s medical history including somatic examination and laboratory tests.

Frequently x-ray exams of the adrenal or pituitary glands are beneficial for locating tumors. These tests assist to find out if excess levels of cortisol are exhibited and why.

 

Radiologic imaging: direct visualization of the endocrine glands. Imaging tests reveal the size and shape of the pituitary and adrenal glands and help determine if a tumor is present.  The most common imaging tests are the computerized tomography (CT) scan and magnetic resonance imaging (MRI).  A CT scan produces a series of x-ray pictures giving a cross-sectional image of a body part.  MRI also produces images of internal organs but without exposing patients to ionizing radiation.

Imaging procedures are used to find a tumor after a diagnosis has been made. Imaging is not used to make the diagnosis of Cushing’s syndrome because benign tumors are commonly found in the pituitary and adrenal glands.  These tumors, sometimes called incidentalomas, do not produce hormones in quantities that are harmful.  They are not removed unless blood tests show they are a cause of symptoms or they are unusually large.  Conversely, pituitary tumors may not be detectable by imaging in almost half of people who ultimately need pituitary surgery for Cushing’s syndrome.

 

24-hour urinary free cortisol level. In this test, a person’s urine is collected several times over a 24-hour period and tested for cortisol.  Levels higher than 50 to 100 micrograms a day for an adult suggest Cushing’s syndrome.  Low-dose dexamethasone suppression test (LDDST). In the LDDST, a person is given a low dose of dexamethasone, a synthetic glucocorticoid, by mouth every 6 hours for 2 days.  Urine is collected before dexamethasone is administered and several times on each day of the test.  A modified LDDST uses a onetime overnight dose.

The normal upper limit varies in different laboratories, depending on which measurement technique is used.

 

Midnight plasma cortisol and late-night salivary cortisol measurements. The midnight plasma cortisol test measures cortisol concentrations in the blood.

 

Dexamethasone-corticotropin-releasing hormone (CRH) test. Some people have high cortisol levels but do not develop the progressive effects of Cushing’s syndrome, such as muscle weakness, fractures, and thinning of the skin.  These people may have pseudo-Cushing’s syndrome, a condition sometimes found in people who have depression or anxiety disorders, drink excess alcohol, have poorly controlled diabetes, or are severely obese.  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.

 

Treatment of Cushings Syndrome:

 Treatment of cushings syndrome is by castigation of the under lying cause. Treatments for Cushing’s syndrome are contrived to pass your body’s cortisol production to normal. By indurate, or even distinctly lowering cortisol levels, you’ll feel evident improvements in your signs and symptoms. Left untreated, however, Cushing’s syndrome can finally induce to death. The treatment choice depend on the cause. For example:

*If a tumour in an adrenal gland is the reason, an operation to withdraw it will cure the condition.

*For adrenal hyperplasia, both adrenal glands may require to be withdraw. You will then require to take lifelong replacement therapy of several adrenal hormones.

*Other tumours in the body that produce ‘ectopic’ ACTH may be able to be removed, depending on the kind of tumour, where it is, etc.

*Medication to block the production or consequence of cortisol may be a choice.

 

Ending line treatments for Cushing syndrome are designed to lower the high level of cortisol in your body. The best treatment for you depends on the cause of the syndrome. Treatment options include:

  • Reducing corticosteroid use. If the cause of Cushing syndrome is long-term use of corticosteroid medications, your doctor may be able to keep your Cushing signs and symptoms under control by reducing the dosage of the drug over a period of time, while still adequately managing your asthma, arthritis or other condition. For many of these medical problems, your doctor can prescribe noncorticosteroid drugs, which will allow him or her to reduce the dosage or eliminate the use of corticosteroids altogether.

Don’t reduce the dose of corticosteroid drugs or stop taking them on your own. Do so only under your doctor’s supervision.

Abruptly discontinuing these medications could lead to deficient cortisol levels. Slowly tapering off corticosteroid drugs allows your body to resume normal cortisol production.

  • If the cause of Cushing syndrome is a tumor, your doctor may recommend complete surgical removal. Pituitary tumors are typically removed by a neurosurgeon, who may perform the procedure through your nose. If a tumor is present in the adrenal glands, lungs or pancreas, the surgeon can remove it through a standard operation or in some cases by using minimally invasive surgical techniques, with smaller incisions.

After the operation, you’ll need to take cortisol replacement medications to provide your body with the correct amount of cortisol. In most cases, you’ll eventually experience a return of normal adrenal hormone production, and your doctor can taper off the replacement drugs.

However, this process can take up to a year or longer. In some instances, people with Cushing syndrome never experience a resumption of normal adrenal function; they then need lifelong replacement therapy.

Radiation therapy. If the surgeon can’t totally remove a pituitary tumor, he or she will usually prescribe radiation therapy to be used in conjunction with the operation. Additionally, radiation may be used for people who aren’t suitable candidates for surgery.

Radiation can be given in small doses over a six-week period, or with a technique called stereotactic radiosurgery (Gamma Knife surgery). In the latter procedure, administered as a single treatment, a large dose of radiation is delivered to the tumor, and the radiation exposure to surrounding tissues is minimized.

  • Medications can be used to control cortisol production when surgery and radiation don’t work. Medications may also be used before surgery in people who have become very sick with Cushing syndrome. Doctors may recommend drug therapy before surgery to improve signs and symptoms and minimize surgical risk.

Medications to control excessive production of cortisol at the adrenal gland include:  ketoconazole (Nizoral), mitotane (Lysodren) and metyrapone (Metopirone).

Mifepristone (Korlym) is approved for people with Cushing syndrome who have type 2 diabetes or glucose intolerance. Mifepristone does not decrease cortisol production, but it blocks the effect of cortisol on your tissues.

Side effects from these medications may include fatigue, nausea, vomiting, headaches, muscle aches, high blood pressure, low potassium and swelling. Some have more serious side effects, such as neurological side effects and liver toxicity.

The newest medication for Cushing’s is pasireotide (Signifor), and it works by decreasing ACTH production from a pituitary tumor. This medication is given as an injection twice daily. It’s recommended if pituitary surgery is unsuccessful or cannot be done.

Side effects are fairly common, and may include diarrhea, nausea, high blood sugar, headache, abdominal pain and fatigue.

In some cases, the tumor or its treatment will cause other hormones produced by the pituitary or adrenal gland to become deficient and your doctor will recommend hormone replacement medications.

If none of these treatment options are appropriate or effective, your doctor may recommend surgical removal of your adrenal glands (bilateral adrenalectomy). This procedure will cure excess production of cortisol, but will require lifelong replacement medications.

Complications are rare with this procedure but could include:

Bruising on your thigh

Ear pain during the procedure

Very rarely, neurological problems

 

QUOTE FOR TUESDAY:

“Cushing’s syndrome is a hormonal disorder caused by prolonged exposure of the body’s tissues to high levels of the hormone cortisol.”

NIH National Institute of Diabetes and Digestive and Kidney Diseases (U.S. Department of Health and Human Services)

 

Part II Cushing Syndrome – signs and symptoms in children and adults with complications!

Symptoms of Cushings Syndrome

 Symptom of cushings syndrome include fat deposits close to the face neck and trunk; weariness; muscular weakness; salt and water retention; acne; leisurely bruising; menstlruall irregularities; and signs (in women) of virilisation, such as increase of the voice, commute in body, shape, loss of scalp hair, and extend in facial and body hair. Complications of cushings syndrome include advanced blood pressure, The symptoms and signs of cushings syndrome induced by a chronic redundant of corticosteroid hormones in the blood. The redundant may be acquired by a tumour of the outer part (cortex) of the adrenal gland, or may be referable to over inspiration of the adrenal glands by a tumour the pituitary gland.

Many children and teenagers with Cushing’s syndrome will exhibit various symptoms of the following:

-extreme weight gain

-growth retardation

-missed periods in teenage girls

-excess hair growth

-acne

-reddish-blue streaks on the skin

-high blood pressure

-tiredness and weakness

-either very early or late puberty

Adults with the disease may also have symptoms of intense weight gain, redundant hair growth, high blood pressure, and skin difficulties. In addition, they may show:

-muscle and bone weakness

-moodiness, irritability, or depression

-sleep disturbances

-high blood sugar

-menstrual disorders in women and diminished fertility in men

Complications include:

-Diabetes (High or Low blood glucose levels)

-Enlargement of pituitary tumor and other complications from the tumor growth

-Fractures due to osteoporosis which are common in older people

-High blood pressure which could be life threatening

-Kidney stones from the increase in cortisol and other chemicals filtered through the kidneys

-Serious infections which could lead to further secondary infections

Stayed tune for Part III on Cushing’s Disease regarding the diagnosing and treatment of Cushing’s Syndrome.

QUOTE FOR MONDAY:

“Too much cortisol can produce some of the hallmark signs of Cushing syndrome — a fatty hump between your shoulders, a rounded face, and pink or purple stretch marks on your skin. Cushing syndrome can also result in high blood pressure, bone loss and, on occasion, type 2 diabetes.”

Mayo Clinic

WHAT IS CUSHING’S SYNDROME

 

Cushing’s syndrome describes the signs and symptoms associated with prolonged exposure to inappropriately high levels of the hormone cortisol. This can be caused by taking glucocorticoid drugs, or diseases that result in excess cortisol, adrenocorticotropic hormone (ACTH), or CRH levels.

Cushing’s syndrome appears when the body’s tissues are display to immoderate levels of cortisol for long periods of time.

 

There are two types of the disease and they are known as exogenous and endogenous.  Exogenous Cushing syndrome is caused by something outside of the body, like when hormones are given to a patient during a RX for another condition.  Endogenous is caused by natural causing problems within the body.  Endogenous is likely to be hereditary and not caused by an outside force like a steroid complex.

 

Causes of Cushing Syndrome

The most common cause of Cushing’s syndrome is exogenous administration of glucocorticoids prescribed by a health care practitioner to treat other diseases (called iatrogenic Cushing’s syndrome). This can be an effect of corticosteroid treatment of a variety of disorders such as asthma and rheumatoid arthritis, or in immunosuppression after an organ transplant.

Administration of synthetic ACTH(adrenocorticotropichormone) is also possible, but ACTH is less often prescribed due to cost and lesser utility. Although rare, Cushing’s syndrome can also be due to the use of medroxyprogesterone   In this form of Cushing’s, the adrenal glands atrophy due to lack of stimulation by ACTH, since glucocorticoids downregulate production of ACTH. Cushing syndrome in childhood usually results from use of glucocorticoid medication.

Endogenous Cushing’s syndrome results from some derangement of the body’s own system of secreting cortisol. Normally, ACTH is released from the pituitary gland when necessary to stimulate the release of cortisol from the adrenal glands.

 

In pituitary Cushing’s, a benign pituitary adenoma secretes ACTH. This is also known as Cushing’s disease and is responsible for 70% of endogenous Cushing’s syndrome.

 

In adrenal Cushing’s, excess cortisol is produced by adrenal gland tumors, hyperplastic adrenal glands, or adrenal glands with nodular adrenal hyperplasia.

 

Tumors outside the normal pituitary-adrenal system can produce ACTH (occasionally with CRH) that affects the adrenal glands. This etiology is called ectopic or paraneoplastic Cushing’s disease and is seen in diseases like small cell lung cancer.

 

Finally, rare cases of CRH-secreting tumors (without ACTH secretion) have been reported, which stimulates pituitary ACTH production.

HYPERLINK “http://en.wikipedia.org/wiki/Cushing%27s_syndrome” \l “cite_note-Voyadzis_JM.2C_Guttman-Bauman_I.2C_Santi_M.2C_Cogen_P._2004_212.E2.80.936

Pseudo-Cushing’s syndrome

 

Elevated levels of total cortisol can also be due to estrogen found in oral contraceptive pills that contain a mixture of estrogen and progesterone, leading to Pseudo-Cushing’s syndrome. Estrogen can cause an increase of cortisol-binding globulin and thereby cause the total cortisol level to be elevated. However, the total free cortisol, which is the active hormone in the body, as measured by a 24 hour urine collection for urinary free cortisol, is normal.

 

Epidemiology

 

Iatrogenic Cushing’s syndrome (caused by treatment with corticosteroids) is the most common form of Cushing’s syndrome.

Stay tune for tomorrow’s topic Part II on signs and symptoms in children and in adults with complications of this syndrome.

 

 

Quote for the Weekend

“In 2014, the 55 U.S. poison control centers provided telephone guidance for nearly 2.2 million human poison exposures.1 That’s about:

  • 6.7 poison exposures/1000 population,
  • 42.6 poison exposures in children younger than 6 years/1000 children,
  • 1 poison exposure reported to U.S. poison control centers every 15 seconds.”

Poison Control – National Capital Poison Center