Categories
Ailmemts & Remedies

Cushing’s syndrome

Alternative Names: Itsenko-Cushing syndrome, hyperadrenocorticism or hypercorticism
Definition:
Cushing’s syndrome is a hormone disorder caused by high levels of cortisol in the blood. This can be caused by taking glucocorticoid drugs, or by tumors that produce cortisol or adrenocorticotropic hormone (ACTH) or CRH

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Cushing’s disease refers to one specific cause of the syndrome, a tumor (adenoma) in the pituitary gland that produces large amounts of ACTH, which in turn elevates cortisol. It is the most common cause of Cushing’s syndrome, responsible for 70% of cases.

This pathology was described by Harvey Cushing in 1932.

Cushing’s syndrome is not confined to humans and is also a relatively common condition in domestic dogs and horses.

Treatments for Cushing’s syndrome can return your body’s cortisol production to normal and noticeably improve your symptoms. The earlier treatment begins, the better your chances for recovery.

Symptoms:
SymptomsMost people with Cushing syndrome will have:

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•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:
•Acne or 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)
•Thinning of the bones, which leads to rib and spine fractures
•Weak muscles

Woman with cushing syndrome often have:

•Excess hair growth on the face, neck, chest, abdomen, and thighs
•Menstrual cycle becomes irregular or stops

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Men may have:
•Decreased fertility
•Decreased or no desire for sex
•Impotence

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Other symptoms that may occur with this disease:
*Mental changes, such as depression, anxiety and irritability or changes in behavior
*Fatigue
*Headache
*High blood pressure
*Increased thirst and urination
*Muscle weakness
*Loss of emotional control
*Cognitive difficulties
*New or worsened high blood pressure
*Glucose intolerance that may lead to diabetes
*Headache
*Bone loss, leading to fractures over time

Iatrogenic Cushing’s syndrome (caused by treatment with corticosteroids) is the most common form of Cushing’s syndrome. The incidence of pituitary tumors may be relatively high, as much as one in five people, but only a minute fraction are active and produce excessive hormones.

Adults with the disease may also have symptoms of extreme weight gain, excess hair growth in women, high blood pressure, and skin problems. In addition, they may show:

*muscle and bone weakness
*osteoporosis
*diabetes mellitus
*hypertension
*moodiness, irritability, or depression
*sleep disturbances
*menstrual disorders such as amenorrhea in women and decreased fertility in men
*baldness
*hypercholesterolemia

Cause:
There are several possible causes of Cushing’s syndrome.

Exogenous vs. endogenous Hormones that come from outside the body are called exogenous; hormones that come from within the body are called endogenous.

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 steroid treatment of a variety of disorders such as asthma and rheumatoid arthritis, or in immunosuppression after an organ transplant. Administration of synthetic ACTH 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.

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.

*Finally, tumors outside the normal pituitary-adrenal system can produce ACTH that affects the adrenal glands. This final etiology is called ectopic or paraneoplastic Cushing’s syndrome and is seen in diseases like small cell lung cancer.

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. 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.

Pathophysiology:
The hypothalamus is in the brain and the pituitary gland sits just below it. The paraventricular nucleus (PVN) of the hypothalamus releases corticotropin-releasing hormone (CRH), which stimulates the pituitary gland to release adrenocorticotropin (ACTH). ACTH travels via the blood to the adrenal gland, where it stimulates the release of cortisol. Cortisol is secreted by the cortex of the adrenal gland from a region called the zona fasciculata in response to ACTH. Elevated levels of cortisol exert negative feedback on the pituitary, which decreases the amount of ACTH released from the pituitary gland. Strictly, Cushing’s syndrome refers to excess cortisol of any etiology. One of the causes of Cushing’s syndrome is a cortisol secreting adenoma in the cortex of the adrenal gland. The adenoma causes cortisol levels in the blood to be very high, and negative feedback on the pituitary from the high cortisol levels causes ACTH levels to be very low. Cushing’s disease refers only to hypercortisolism secondary to excess production of ACTH from a corticotrophic pituitary adenoma. This causes the blood ACTH levels to be elevated along with cortisol from the adrenal gland. The ACTH levels remain high because a tumor causes the pituitary to be unresponsive to negative feedback from high cortisol levels.

Cushing’s Syndrome was also the first autoimmune disease identified in humans.

Diagnosis:-
When Cushing’s syndrome is suspected, either a dexamethasone suppression test (administration of dexamethasone and frequent determination of cortisol and ACTH level), or a 24-hour urinary measurement for cortisol offer equal detection rates. Dexamethasone is a glucocorticoid and simulates the effects of cortisol, including negative feedback on the pituitary gland. When dexamethasone is administered and a blood sample is tested, high cortisol would be indicative of Cushing’s syndrome because there is an ectopic source of cortisol or ACTH (e.g.: adrenal adenoma) that is not inhibited by the dexamethasone. A novel approach, recently cleared by the US FDA, is sampling cortisol in saliva over 24 hours, which may be equally sensitive, as late night levels of salivary cortisol are high in Cushingoid patients. Other pituitary hormone levels may need to be ascertained. Performing a physical examination to determine any visual field defect may be necessary if a pituitary lesion is suspected, which may compress the optic chiasm causing typical bitemporal hemianopia.

When any of these tests are positive, CT scanning of the adrenal gland and MRI of the pituitary gland are performed to detect the presence of any adrenal or pituitary adenomas or incidentalomas (the incidental discovery of harmless lesions). Scintigraphy of the adrenal gland with iodocholesterol scan is occasionally necessary. Very rarely, determining the ACTH levels in various veins in the body by venous catheterization, working towards the pituitary (petrosal sinus sampling) is necessary.

Mnemonic:
C – Central obesity, Cervical fat pads, Collagen fibre weakness, Comedones (acne)
U – Urinary free cortisol and glucose increase
S – Striae, Suppressed immunity
H – Hypercortisolism, Hypertension, Hyperglycemia, Hypercholesterolemia, Hirsutism
I – Iatrogenic (Increased administration of corticosteroids)
N – Noniatrogenic (Neoplasms)
G – Glucose intolerance, Growth retardation

Treatment:-
Most Cushing’s syndrome cases are caused by steroid medications (iatrogenic). Consequently, most patients are effectively treated by carefully tapering off (and eventually stopping) the medication that causes the symptoms.

If an adrenal adenoma is identified it may be removed by surgery. An ACTH-secreting corticotrophic pituitary adenoma should be removed after diagnosis. Regardless of the adenoma’s location, most patients will require steroid replacement postoperatively at least in the interim as long-term suppression of pituitary ACTH and normal adrenal tissue does not recover immediately. Clearly, if both adrenals are removed, replacement with hydrocortisone or prednisolone is imperative.

In those patients not suitable for or unwilling to undergo surgery, several drugs have been found to inhibit cortisol synthesis (e.g. ketoconazole, metyrapone) but they are of limited efficacy.

Removal of the adrenals in the absence of a known tumor is occasionally performed to eliminate the production of excess cortisol. In some occasions, this removes negative feedback from a previously occult pituitary adenoma, which starts growing rapidly and produces extreme levels of ACTH, leading to hyperpigmentation. This clinical situation is known as Nelson’s syndrome.

Lifestyle and home remedies:-

The length of your recovery from Cushing’s syndrome will depend on the severity and cause of your condition. Remember to be patient. You didn’t develop Cushing’s syndrome overnight and your symptoms won’t disappear overnight, either. In the meantime, these tips may help you on your journey back to health.

*Increase activities slowly. You may be in such a hurry to get your old self back that you push yourself too hard too fast, but your weakened muscles need a slower approach. Work up to a reasonable level of exercise or activity that feels comfortable without overdoing it. You’ll improve little by little, and your persistence will be rewarded.

*Eat sensibly. Nutritious, wholesome foods provide a good source of fuel for your recovering body and can help you lose the extra pounds that you gained from Cushing’s syndrome. Make sure you’re getting enough calcium and vitamin D. Taken together, they help your body absorb calcium, which can help strengthen your bones, counteracting the bone density loss that often occurs with Cushing’s syndrome.

*Monitor your mental health. Depression can be a side effect of Cushing’s syndrome, but it can also persist or develop after treatment begins. Don’t ignore your depression or wait it out. Seek help promptly from your doctor or a therapist if you’re depressed, overwhelmed or having difficulty coping during your recovery.

*Gently soothe aches and pains. Hot baths, massages and low-impact exercises, such as water aerobics and tai chi, can help alleviate some of the muscle and joint pain that accompanies Cushing’s syndrome recovery.

*Exercise your brain. If you’re recovering from any cognitive difficulties as a result of Cushing’s syndrome, mental exercises, such as math problems and crossword puzzles, may improve your brain function.

Coping and support:-
Support groups can be valuable in dealing with Cushing’s syndrome and recovery. They bring you together with other people who are coping with the same kinds of challenges, along with their families and friends, and offer a setting in which youe can share common problems.

Ask your doctor about support groups in your community. Your local health department, public library and telephone book and the Internet also may be good sources to find a support group in your area.

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.

Disclaimer: This information is not meant to be a substitute for professional medical advise or help. It is always best to consult with a Physician about serious health concerns. This information is in no way intended to diagnose or prescribe remedies.This is purely for educational purpose.

Resources:
http://en.wikipedia.org/wiki/Cushing’s_syndrome
http://www.mayoclinic.com/health/cushings-syndrome/DS00470
http://www.nlm.nih.gov/medlineplus/ency/article/000410.htm
http://www.bbc.co.uk/health/physical_health/conditions/cushing1.shtml

Cushing’s Syndrome


http://www.potbellysyndrome.com/documents/083EFB330BDDC27C6EEC8354AFFA139607633EB6.html
http://www.wrongdiagnosis.com/c/cushings_disease/book-diseases-7a.htm

http://www.nature.com/eye/journal/v20/n6/fig_tab/6701956f4.html

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Categories
Ailmemts & Remedies

Amyloidosis

Alternative Names: Amyloid – primary

Definition:
In medicine, amyloidosis refers to a variety of conditions in which amyloid proteins are abnormally deposited in organs and/or tissues. A protein is described as being amyloid if, due to an alteration in its secondary structure, it takes on a particular aggregated insoluble form similar to the beta-pleated sheet.  Symptoms vary widely depending upon the site of amyloid deposition. Amyloidosis may be inherited or acquired.

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The collection of these abnormal proteins interferes with the normal functioning of the organ affected.

Since there are more than 20 different proteins that may form amyloid, there are also many different types of amyloidosis.

Classification of amyloid:
The modern classification of amyloid disease tends to use an abbreviation of the protein that makes the majority of deposits, prefixed with the letter A. For example amyloidosis caused by transthyretin is termed “ATTR.” Deposition patterns vary between patients but are almost always composed of just one amyloidogenic protein. Deposition can be systemic (affecting many different organ systems) or organ-specific. Many amyloidoses are inherited, due to mutations in the precursor protein. Other forms are due to different diseases causing overabundant or abnormal protein production – such as with over production of immunoglobulin light chains in multiple myeloma (termed AL amyloid), or with continuous overproduction of acute phase proteins in chronic inflammation (which can lead to AA amyloid).

Out of the approximately 60 amyloid proteins that have been identified so far,  at least 36 have been associated in some way with a human disease.

Amyloidosis is rare, being diagnosed in between one and five in every 100,000 people every year. It’s more common in older people and is also slightly more common in men than in women.

Causes:
The cause of primary amyloidosis is unknown, but the condition is related to abnormal production of antibodies by a type of immune cell called plasma cells.

The symptoms depend on the organs affected by the deposits. These organs can include the tongue, intestines, skeletal and smooth muscles, nerves, skin, ligaments, heart, liver, spleen, and kidneys.

Primary amyloidosis can result in conditions that include:

•Carpal tunnel syndrome
•Gastrointestinal reflux (GERD)
•Heart muscle damage (cardiomyopathy)
•Kidney failure
•Malabsorption
The deposits build up in the affected organs, causing them to become stiff, which decreases their ability to function.

Risk factors have not been identified. Primary amyloidosis is rare. It is similar to multiple myeloma, and is treated the same way.

Symptoms:

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•Enlarged tongue
•Fatigue
•Irregular heart rhythm
•Numbness of hands and feet
•Shortness of breath
•Skin changes
•Swallowing difficulties
•Swelling in the arms and legs
•Weak hand grip
•Weight loss

Additional symptoms that may be associated with this disease:
•Clay-colored stools
•Decreased urine output
•Diarrhea
•Hoarseness or changing voice
•Joint pain
•Other tongue problems
•Weakness

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Diagnosis:
Exams and Tests
Your doctor may discover that you have an enlarged liver or spleen.

If specific organ damage is suspected, your doctor may order tests to confirm amyloidosis of that organ. For example:

•Abdominal ultrasound may reveal a swollen liver or spleen.
•An abdominal fat pad biopsy, rectal mucosa biopsy, or a bone marrow biopsy can help confirm the diagnosis.
•A heart evaluation, including an ECG,may reveal arrhythmias, abnormal heart sounds, or signs of congestive heart failure. An echocardiogram shows poor motion of the heart wall, due to a stiff heart muscle.
•A carpal tunnel syndrome evaluation may show that hand grips are weak.Nerve conduction velocity shows abnormalities.
•Kidney function tests may show signs of kidney failure or too much protein in the urine ( nephrotic syndrome).
?BUN level is increased.
?Serum creatinine is increased.
?Urinalysis shows protein, casts, or fat bodies.

This disease may also alter the results of the following tests:
•Bence-Jones protein (quantitative)
•Carpal tunnel biopsy
•Gum biopsy
•Immunoelectrophoresis – serum
•Myocardial biopsy
•Nerve biopsy
•Quantitative immunoglobulins
•Tongue biopsy
•Urine protein

Treatment:
It isn’t always easy to treat amyloidosis, and there is no treatment yet that specifically targets the amyloid depositing in the tissues. In cases where it’s secondary to another problem (AA amyloidosis), such as rheumatoid arthritis, treating that original problem may stop the progress of amyloidosis or may even reverse it.

In cases of primary amyloidosis (AL amyloidosis), chemotherapy drugs may be given to suppress production of new amyloid and cause regression of existing amyloid deposits.

In secondary amyloidosis, aggressive treatment of the underlying disease can improve symptoms and/or slow progression of disease. Complications such as heart failure, kidney failure, and other problems can sometimes be treated as necessary.

Occasionally, transplantation of a damaged organ is necessary. However, even after this has been carried out the new organ may become affected by amyloidosis.

Treatment may also be aimed at supporting the function of damaged tissues and treating complications such as heart or kidney failure.

Overall, many types of amyloidosis follow a steadily progressive course and may prove fatal within a year or two.

Prognosis :
The severity of the disease depends upon the organs affected. Heart and kidney involvement may lead to organ failure and death. Systemic involvement is associated with death within 1 to 3 years.

Possible Complications:
•Congestive heart failure
•Death
•Endocrine failure (hormonal disorder)
•Kidney failure
•Respiratory failure

Prevention : There is no known prevention.

Disclaimer: This information is not meant to be a substitute for professional medical advise or help. It is always best to consult with a Physician about serious health concerns. This information is in no way intended to diagnose or prescribe remedies.This is purely for educational purpose.

Resources:
http://www.bbc.co.uk/health/physical_health/conditions/amyloidosis1.shtml
http://www.nlm.nih.gov/medlineplus/ency/article/000533.htm
http://en.wikipedia.org/wiki/Amyloidosis

http://health.allrefer.com/pictures-images/amyloidosis-on-the-face.html

http://health.allrefer.com/health/cardiac-amyloidosis-dilated-cardiomyopathy.html

http://morningreporttgh.blogspot.com/2010/04/amyloidosis.html

http://gsm.utmck.edu/research/HICP/overview.cfm

http://www.pathologyatlas.ro/amyloidosis-kidney-pathology.php

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Categories
News on Health & Science

Hope Over High Blood Pressure Jab

A four-monthly jab may one day replace the need to regularly take pills to control blood pressure, scientists say.

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High blood pressure is a risk factor for heart disease

A team from the Swiss biotechnology firm Cytos found the vaccine against a hormone in the blood significantly cut blood pressure, the Lancet reported.

The jab was tested on 72 patients with high blood pressure and it was found to work without serious side-effects.

The researchers and independent experts said the findings were promising but large-scale trials were now needed.

High blood pressure, which affects a quarter of all adults, doubles the risk of dying from heart disease or stroke and is blamed for 60,000 deaths a year in UK.

Those who are being treated for it often have to take a daily course of pills to keep it under control.

But many people do not keep to their treatment regimes as people with high blood pressure do not display symptoms.

The researchers believe the vaccine, which works against the hormone angiotensin, which causes blood vessels to constrict and increase blood pressure, may offer a simple alternative.

They tested two different doses of the vaccine – 300 microgrammes and 100 microgrammes – as well as a dummy vaccine during the 14-week trial.

Resistance

The jabs were given at the start, and after four weeks and 12 weeks – enough to give a patient four-month resistance.

Neither dose significantly lowered blood pressure at night.

But during the day the larger dose significantly lowered blood pressure, especially during the late morning peak when blood pressure is known to increase.

And, importantly, the vaccine did not have any serious side-effects with the worst being mild flu-like symptoms.

Lead researcher Dr Martin Bachmann said the vaccine could offer a much more simple way of controlling blood pressure and could be administered during regular visits to the doctor.

“Such a regimen is likely to promote adherence to treatment, but will need to be supported by clinical data.”

Professor Jeremy Pearson, of the British Heart Foundation, agreed more research was needed, but described the results as promising.

“Immunisation may be of particular benefit to people who find it difficult to stick to high blood pressure medication, but there is still a long way to go before this approach replaces the highly-effective current treatments.

“Looking after your heart through regular exercise, cutting down on salt, and only drinking in moderation remain the best ways in which we can prevent high blood pressure.”

Other firms are also known to be testing blood pressure vaccines.

Click to see also:->
High blood pressure set to soar

Hypertension trial stopped early

Finger test ‘spots heart disease’

Water ‘helps low blood pressure’

Warning over blood pressure drugs

Sleep drug lowers blood pressure

Gene clues to high blood pressure
Sources: BBC NEWS:7Th. March. ’08