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Ailmemts & Remedies

Graves’ disease

Alternative Names :Toxic diffuse goiter, thyrotoxicosis,diffuse thyrotoxic goiter

Definition:
Graves’ disease is the most common cause of hyperthyroidism . Hyperthyroidism is an autoimmune disorder that occurs when the thyroid gland makes more thyroid hormone than the body needs.

The thyroid is a small, butterfly-shaped gland in the front of the neck below the larynx, or voice box. The thyroid gland makes two thyroid hormones, triiodothyronine (T3) and thyroxine (T4). Thyroid hormones affect metabolism, brain development, breathing, heart and nervous system functions, body temperature, muscle strength, skin dryness, menstrual cycles, weight, and cholesterol levels.


Click to see the picture

Thyroid hormone production is regulated by another hormone called thyroid-stimulating hormone (TSH), which is made by the pituitary gland located in the brain.

Graves’ disease is an autoimmune disorder, meaning the body’s immune system acts against its own healthy cells and tissues. In Graves’ disease, the immune system makes antibodies called thyroid-stimulating immunoglobulin (TSI) that attach to thyroid cells. TSI mimics the action of TSH and stimulates the thyroid to make too much thyroid hormone. Sometimes the antibodies can instead block thyroid hormone production, leading to a confusing clinical picture. The diagnosis and treatment of Graves’ disease is often performed by an endocrinologist—a doctor who specializes in the body’s hormone-secreting glands.

Graves’ disease is rarely life-threatening. Although it may develop at any age and in either men or women, Graves’ disease is more common in women and usually begins after age 20.

Recent studies in England put the incidence of Graves’ disease at 1 to 2 cases per 1,000 population per year (in England). It occurs much more frequently in women than in men. The disease frequently presents itself during early adolescence or begins gradually in adult women, often after childbirth, and is progressive until treatment. It has a powerful hereditary component.

Graves’ disease tends to be more severe in men, even though it is rarer. It appears less likely to go into permanent remission and the eye disease tends to be more severe, but men are less likely to have large goitres. In a statistical study of symptoms and signs of 184 thyrotoxic patients (52 men, 132 women), the male patients were somewhat older than the females, and there were more severe cases among men than among women. Cardiac symptoms were more common in women, even though the men were older and more often had a severe form of the disease; palpitations and dyspnea were more common and severe in women.

Cigarette smoking, which is associated with many autoimmune diseases, raises the incidence of Graves’ ophthalmopathy 7.7-fold.

There’s no way to stop your immune system from attacking your thyroid gland, but treatments for Graves’ disease can ease symptoms and decrease the production of thyroxine.

Symptoms:
Graves’ disease symptoms may include:

*Anxiety

*Difficulty sleeping
*Fatigue
*A rapid or irregular heartbeat
*A fine tremor of your hands or fingers
*An increase in perspiration
*Sensitivity to heat
*Weight loss, despite normal food intake
*Brittle hair
*Enlargement of your thyroid gland (goiter)
*Change in menstrual cycles
*Frequent bowel movements
*Increased appetite
*Diarrhoea
*Tremor and shaking
*Irritability and emotional upsets
*Profuse sweating
*Dislike of hot weather
*Itching, reddening and thickening of the skin, typically over the shins

Graves’ ophthalmopathy
It’s also fairly common for your eyes to exhibit mild signs of a condition known as Graves’ ophthalmopathy. In Graves’ ophthalmopathy, your eyeballs bulge out past their protective orbit (exophthalmos). This occurs as tissues and muscles behind your eyes swell and cause your eyeballs to move forward. Because your eyes may be pushed so far forward, the front surface of your eyes can become dry. Cigarette smokers with Graves’ disease are five times more likely than nonsmokers to develop Graves’ ophthalmopathy. This is possibly because smoking inhibits the absorption of anti-thyroid medication that is used to treat Graves’ disease.

Graves’ ophthalmopathy may cause these mild signs and symptoms:

*Excess tearing and sensation of grit or sand in either or both eyes
*Reddened or inflamed eyes
*Widening of the space between your eyelids
*Swelling of the lids and tissues around the eyes
*Light sensitivity
.
Less often, Graves’ ophthalmopathy can produce these serious signs and symptoms:
*Ulcers on the cornea
*Double vision
*Limited eye movements
*Blurred or reduced vision

There may also be a goitre (or swelling of the thyroid gland in the neck) and swelling of the tissues over the front of the shins.

Cause:
The trigger for autoantibody production is unknown.

Since Graves’ disease is an autoimmune disease which appears suddenly, often quite late in life, it is thought that a viral or bacterial infection may trigger antibodies which cross-react with the human TSH receptor (a phenomenon known as antigenic mimicry, also seen in some cases of type I diabetes)[citation needed]. One possible culprit is the bacterium Yersinia enterocolitica (not the same as Yersinia pestis, the agent of bubonic plague). However, although there is indirect evidence for the structural similarity between the bacterium and the human thyrotropin receptor, direct causative evidence is limited.  Yersinia seems not to be a major cause of this disease, although it may contribute to the development of thyroid autoimmunity arising for other reasons in genetically susceptible individuals.  It has also been suggested that Y. enterocolitica infection is not the cause of auto-immune thyroid disease, but rather is only an associated condition; with both having a shared inherited susceptibility. More recently the role for Y. enterocolitica has been disputed.

Some of the eye symptoms of hyperthyroidism are believed to result from heightened sensitivity of receptors to sympathetic nervous system activity, possibly mediated by increased alpha-adrenergic receptors in some tissues.

Like most auto-immune conditions, women seem to be far more susceptible. Graves is up to eight times more common in women than men.

Some people may have a genetic predisposition to develop TSH receptor autoantibodies. HLADR (especially DR3) appears to play a significant role.

Risk Factors:
It’s known there are links between autoimmune conditions, so Graves’ disease is linked to insulin-dependent diabetes and pernicious anaemia (which are classed as autoimmune conditions). So when a person has one of these, they or members of their family may be at increased risk of developing another.

There’s also a genetic influence contributing to Graves’ disease and it can run in families.

Diagnosis:-
The onset of Graves’ disease symptoms is often insidious: the intensity of symptoms can increase gradually for a long time before the patient is correctly diagnosed with Graves’ disease, which may take months or years. (Not only Graves’ disease, but most endocrinological diseases have an insidious, subclinical onset.) One study puts the average time for diagnosis at 2.9 years, having observed a range from 3 months to 20 years in their sample population. A 1996 study offers a partial explanation for this generally late diagnosis, suggesting that the psychiatric symptoms (due to the hyperthyroidism) appeared to result in delays in seeking treatment as well as delays in receiving appropriate diagnosis. Also, earlier symptoms of nervousness, hyperactivity, and a decline in school performance, may easily be attributed to other causes.[citation needed] Many symptoms may occasionally be noted, at times, in otherwise healthy individuals who do not have thyroid disease (e.g., everyone feels anxiety and tension to some degree), and many thyroid symptoms are similar to those of other diseases. Thus, clinical findings may be full blown and unmistakable or insidious and easily confused with other disorders. The results of overlooking the thyroid can however be very serious.  Also noteworthy and problematic, is that in a 1996 survey study respondents reported a significant decline in memory, attention, planning, and overall productivity from the period 2 years prior to Graves’ symptoms onset to the period when hyperthyroid.[28] Also, hypersensitivity of the central nervous system to low-grade hyperthyroidism can result in an anxiety disorder before other Graves’ disease symptoms emerge. E.g., panic disorder has been reported to precede Graves’ hyperthyroidism by 4 to 5 years in some cases, although it is not known how frequently this occurs.

English: Photograph showing a classic finding ...
English: Photograph showing a classic finding of Graves’ Disease, proptosis and lid retraction. (Photo credit: Wikipedia)

The resulting hyperthyroidism in Graves’ disease causes a wide variety of symptoms. The two signs that are truly ‘diagnostic’ of Graves’ disease (i.e., not seen in other hyperthyroid conditions) are exophthalmos (protuberance of one or both eyes) and pretibial myxedema, a rare skin disorder with an occurrence rate of 1-4%, that causes lumpy, reddish skin on the lower legs. Graves’ disease also causes goitre (an enlargement of the thyroid gland) that is of the diffuse type (i.e., spread throughout the gland). This phenomenon also occurs with other causes of hyperthyroidism, though Graves’ disease is the most common cause of diffuse goitre. A large goitre will be visible to the naked eye, but a smaller goitre (very mild enlargement of the gland) may be detectable only by physical exam. Occasionally, goitre is not clinically detectable but may be seen only with CT or ultrasound examination of the thyroid.

A highly suggestive symptom of hyperthyroidism, is a change in reaction to external temperature. A hyperthyroid person will usually develop a preference for cold weather, a desire for less clothing and less bed covering, and a decreased ability to tolerate hot weather. When thyroid disease runs in the family, the physician should be particularly wary: studies of twins suggest that the genetic factors account for 79% of the liability to the development of Graves’ disease (whereas environmental factors account presumably for the remainder).  Other nearly pathognomonic signs of hyperthyroidism are excessive sweating, high pulse during sleep, and a pattern of weight loss with increased appetite (although this may also occur in diabetes mellitus and malabsorption or intestinal parasitism).

Hyperthyroidism in Graves’ disease is confirmed, as with any other cause of hyperthyroidism, by a blood test. Elevated blood levels of the principal thyroid hormones (i.e. free T3 and T4), and a suppressed thyroid-stimulating hormone (low due to negative feedback from the elevated T3 and T4), point to hyperthyroidism

However, a 2007 study makes clear that diagnosis depends to a considerable extent on the position of the patient’s unique set point for T4 and T3 within the laboratory reference range (an important issue which is further elaborated below).

Differentiating Graves’ hyperthyroidism from the other causes of hyperthyroidism (thyroiditis, toxic multinodular goiter, toxic thyroid nodule, and excess thyroid hormone supplementation) is important to determine proper treatment. Thus, when hyperthyroidism is confirmed, or when blood results are inconclusive, thyroid antibodies should be measured. Measurement of thyroid stimulating immunoglobulin (TSI) is the most accurate measure of thyroid antibodies. They will be positive in 60 to 90% of children with Graves’ disease. If TSI is not elevated, then a radioactive iodine uptake should be performed; an elevated result with a diffuse pattern is typical of Graves’ disease. Biopsy to obtain histological testing is not normally required but may be obtained if thyroidectomy is performed.

Treatment :
Treatment aims to:
•Keep thyroid hormone levels in the normal range
•Prevent eye problems (which can result from exposure of the delicate eye tissues in Graves’ opthalmopathy) – this can be very difficult and eye symptoms may persist even when treatments work well to keep thyroid hormone levels within the normal range.

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Treatment for the raised hormone levels can include:
•Drugs for immediate and then long-term control
•Surgery to remove part of the thyroid gland
•Radioactive iodine treatment (RAI)

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In recent years, efforts have been made to find a dose of RAI that will give a good cure rate for thyrotoxicosis without leading to underactivity (known as hypothyroidism). However, this has proved difficult and hypothyroidism remains a side-effect of the treatment, affecting as many as 20 per cent of those treated within the first couple of years after treatment, and three to five per cent more each year after that.

Those affected may need lifelong supplements of thyroid hormones.

Treatment for the eye problems includes:
•Drug treatments and eye drops to reduce swelling and close lids
•Steroids, especially if the eye muscles are paralysed or the swelling is very bad
•Surgery to reduce swelling or closed lids
•Radiotherapy is sometimes used in difficult cases.

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Neuropsychiatric symptoms:
A substantial proportion of patients have an altered mental state, even after successful treatment of hyperthyroidism. When psychiatric disorders remain after restoration of euthyroidism and after treatment with beta blockers, specific treatment for the psychiatric symptoms, especially psychotropic drugs, may be needed.[10] A literature study concluded in 2006, found that, after being diagnosed with Graves’ hyperthyroidism, approximately one-third of patients are prescribed psychotropic drugs. Sometimes these drugs are given to treat mental symptoms of hyperthyroidism, sometimes to treat mental symptoms remaining after amelioration of hyperthyroidism, and sometimes when the diagnosis of Graves’ hyperthyroidism has been missed and the patient is treated as having a primary psychiatric disorder. There are no systematic data on the general efficacy of psychotropic drugs in the treatment of mental symptoms in patients with hyperthyroidism, although many reports describe the use of individual agents.  De Groot mentions that a mild sedative or tranquilizer is often helpful.  German research of 2004 reported that 35 percent of treated Graves’ disease patients (with normal thyroid tests for at least six months after treatment), suffered from psychological distress, and had high levels of anxiety. Almost all these patients had clear-cut depression

General measurements:
Graves’ disease patients are nutritionally depleted in proportion to the duration and severity of their illness. Until metabolism is restored to normal, and for some time afterward, caloric and protein requirements may be well above normal. Specific deficiencies may exist, and multivitamin supplementation is indicated. The intake of calcium should be above normal. All in all, the physician should pay heed to the patient’s emotional needs, as well as to his or her requirements for rest, nutrition, and specific (anti)thyroid medication

Prognosis:
The disease typically begins gradually, and is progressive unless treated. If left untreated, more serious complications could result, including bone loss and fractures, inanition, birth defects in pregnancy, increased risk of a miscarriage. Graves disease is often accompanied by an increase in heart rate, which may lead to cardiovascular damage and further heart complications including loss of the normal heart rhythm (atrial fibrillation), which may lead to stroke. If the eyes are bulging severely enough that the lids do not close completely at night, severe dryness will occur with a very high risk of a secondary corneal infection which could lead to blindness. Pressure on the optic nerve behind the globe can lead to visual field defects and vision loss as well. In severe thyrotoxicosis, a condition frequently referred to as thyroid storm, the neurologic presentations are more fulminant, progressing if untreated through an agitated delirium to somnolence and ultimately to coma. All in all, untreated Graves’ disease can lead to significant morbidity, disability and even death. However, the long-term history also includes spontaneous remission in some cases and eventual spontaneous development of hypothyroidism if autoimmune thyroiditis coexists and destroys the thyroid gland.

When effective thyroid treatment is begun, the general response is quite favorable: physical symptoms resolve, vitality returns and the mental processes become efficient again. However, symptom relief is usually not immediate and is achieved over time as the treatments take effect and thyroid levels reach stability. In addition, not all symptoms may resolve at the same time. Prognosis also depends on the duration and severity of the disease before treatment. Swedish research of 2005 reports a lower quality of life for 14 to 21 years after treatment of Graves’ disease, with lower mood and lower vitality, regardless of the choice of treatment.

Remission and relapses:
A literature study in 2006 found that patients who have residual mental symptoms have a significantly higher chance of relapse of hyperthyroidism. Patients with recurrent Graves’ hyperthyroidism, compared with patients in remission and healthy subjects, had significantly higher scores on scales related to depression and anxiety, as well as less tolerance of stress.  According to a 2010 publication, a total thyroidectomy offers the best chance of preventing recurrent hyperthyroidism.

Mental impairment:
A literature review in 2006, whilst noting methodology issues in the consistency of Graves’ disease diagnostic criteria, found many reports about residual complaints in patients who were euthyroid after treatment with a high prevalence of anxiety disorders and bipolar disorder, as well as elevated scores on scales of anxiety, depression and psychological distress.  Bunevicius et al. point out that this “substantial mental disability” is more severe in patients with residual hyperthyroidism but is present even in euthyroid patients. Delay in therapy markedly worsens the prognosis for recovery, but complications can be prevented by early treatment.  In rare cases, patients will experience psychosis-like symptoms only after they have been treated for hypo- or hyperthyroidism, due to a rapid normalisation of thyroid hormone levels in a patient who has partly adapted to abnormal values.

Thyroid replacement treatment after thyroidectomy or radioiodine:
Several studies find a high frequency of TSH level abnormalities in patients who take thyroid hormone supplemenation for long periods of time, and stress the importance of periodic assessment of serum TSH.

Possible Complications:-
*Complications from surgery, including:
*Hoarseness from damage to the nerve leading to the voice box
*Low calcium levels from damage to the parathyroid glands (located near the thyroid gland)
*Scarring of the neck

*Eye problems (called Graves ophthalmopathy or exophthalmos)

•Heart-related complications, including:
*Rapid heart rate
*Congestive heart failure (especially in the elderly)
*Atrial fibrillation

*Thyroid crisis (thyrotoxic storm), a severe worsening of overactive thyroid gland symptoms
*Increased risk for osteoporosis, if hyperthyroidism is present for a long time

•Complications related to thyroid hormone replacement
*If too little hormone is given, fatigue, weight gain, high cholesterol, depression, physical sluggishness, and other symptoms of hypothyroidism can occur
*If too much hormone is given, symptoms of hyperthyroidism will return

Lifestyle and Home Remedies:
For Graves’ ophthalmopathy
These steps may make your eyes feel better if you have Graves’ ophthalmopathy:

*Apply cool compresses to your eyes. The added moisture may soothe your eyes.

*Wear sunglasses. When your eyes protrude, they’re more vulnerable to ultraviolet rays and more sensitive to bright light. Wearing sunglasses that wrap around the sides of your head will lessen the irritation of your eyes from the wind.

*Use lubricating eyedrops. Eyedrops may relieve the dry, scratchy sensation on the surface of your eyes. At night, a paraffin-based gel such as Lacri-Lube can be applied.

*Elevate the head of your bed. Keeping your head higher than the rest of your body lessens fluid accumulation in the head and may relieve the pressure on your eyes.

For Graves’ dermopathy
If the disease affects your skin (Graves’ dermopathy), use over-the-counter creams or ointments containing hydrocortisone to relieve swelling and reddening. In addition, using compression wraps on your legs may help.

Coping and support:
If you have Graves’ disease, make your mental and physical well-being a priority. Eating well and exercising can enhance the improvement in some symptoms while being treated and help you feel better in general. For example, because your thyroid controls your metabolism, you may have a tendency to gain weight when the hyperthyroidism corrects. Brittle bones can also occur with Graves’ disease and weight-bearing exercises can help maintain bone density.

Try to ease stress as much as you can, as stress possibly contributes to the development of Graves’ disease. Listening to music, taking a warm bath or walking can help relax you and put you in a better frame of mind. Partner with your doctor to construct a plan that incorporates good nutrition, exercise and relaxation into your daily routine.

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/gravesdisease.shtml
http://www.nlm.nih.gov/medlineplus/ency/article/000358.htm
http://en.wikipedia.org/wiki/Graves’_disease
http://www.mayoclinic.com/health/graves-disease/DS00181

http://www.nlm.nih.gov/medlineplus/ency/imagepages/17067.htm

http://endocrine.niddk.nih.gov/pubs/graves/#symptoms

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Ailmemts & Remedies

Behçet’s Disease

Definition:
Behcet’s disease is a rare, chronic disorder involving inflammation of blood vessels throughout the body. It is marked by recurrent oral and genital ulcers and eye inflammation.It is  an autoimmune response where the immune system turns on the body, causes inflammation of parts of the body. In particular, small blood vessels around the body become inflamed, a condition known as vasculitis.

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The cause of Behcet’s remains unknown, but it’s often preceded by a viral infection, for example, which may trigger the autoimmune process, causing the body to attack its own blood vessels and making them inflamed. Experts in the field of Behcet’s research agree the causes may be genetic.

Life for people with Behcet’s is made more difficult because of misunderstandings about the illness. With the appearance of ulcers on the mouth and genitals, it’s often incorrectly assumed the condition is infectious and sexually transmitted – it’s not either of these.

You may click to see more pictures  of Bechcet’s disesse

The disease was first described in 1937 by Dr. Hulusi Behçet, a dermatologist in Turkey. Behçet’s disease is now recognized as a chronic condition that causes canker sores or ulcers in the mouth and on the genitals, and inflammation in parts of the eye. In some people, the disease also results in arthritis (swollen, painful, stiff joints), skin problems, and inflammation of the digestive tract, brain, and spinal cord.

Sign & Symptoms;
Integumentary system (Skin and mucosa):
Nearly all patients present with some form of painful oral mucocutaneous ulcerations in form of aphthous ulcers or non-scarring oral lesions. The oral lesions are similar to those found in inflammatory bowel disease and can be relapsing. Painful genital ulcerations usually develop on the vulva and the scrotum and cause scarring in 75% of the patients. Additionally, patients may present with erythema nodosum, cutaneous pustular vasculitis, and lesions similar to pyoderma gangrenosum.

Ocular system (eyes):…..
Inflammatory eye disease can develop early in the disease course and lead to permanent vision loss in 20% of the cases.  Ocular involvement can be in form of posterior uveitis, anterior uveitis, or retinal vasculitis. Anterior uveitis presents with painful eyes, conjuctival redness, hypopyon, and decreased visual acuity, while posterior uveitis presents with painless decreased visual acuity and visual field floaters. A rare form of ocular (eye) involvement in this syndrome is retinal vasculitis which presents with painless decrease of vision with possibility of floaters or visual field defects.

Gastrointestinal tract (bowels)
GI manifestations include abdominal pain, nausea, diarrhea with or without blood and often involves the ileocecal valve.

Pulmonary (lungs)
Lung involvement is typically in form of hemoptysis, pleuritis, cough, fever, and in severe cases can be life threatening if the outlet pulmonary artery develops an aneurysm which ruptures causing severe vascular collapse and death from bleeding in the lungs.

Musculoskeletal system (muscle, joint)

Arthralgia is seen in up to half of patients, and is usually a non-erosive poly or oligoarthritis of primarily the large joints of the lower extremities.

Neurological system:
Neurological involvements range from aseptic meningitis, to vascular thrombosis such as dural sinus thrombosis and or organic brain syndrome manifesting with confusion, seizures, and memory loss. They oftern appear late in the progression of the disease but are associated with a poor prognosis.

Causes:
The exact cause of Behçet’s disease is unknown. Most symptoms of the disease are caused by inflammation of the blood vessels. Inflammation is a characteristic reaction of the body to injury or disease and is marked by four signs: swelling, redness, heat, and pain. Doctors think that an autoinflammatory reaction may cause the blood vessels to become inflamed, but they do not know what triggers this reaction. Under normal conditions, the immune system protects the body from diseases and infections by killing harmful “foreign” substances, such as germs, that enter the body. In an autoimmune reaction, the immune system mistakenly attacks and harms the body’s own tissues.

Behçet’s disease is not contagious; it is not spread from one person to another. Researchers think that two factors are important for a person to get Behçet’s disease. First, it is believed that abnormalities of the immune system make some people susceptible to the disease. Scientists think that this susceptibility may be inherited; that is, it may be due to one or more specific genes. Second, something in the environment, possibly a bacterium or virus, might trigger or activate the disease in susceptible people.

Diagnosis:
There is no specific pathological testing or technique available for the diagnosis of the disease, although the International Study Group criteria for the disease are highly sensitive and specific, involving clinical criteria and a pathergy test.[2][3] Behçet’s disease has a high degree of resemblance to diseases that cause mucocutaneous lesions such as Herpes simplex labialis, and therefore clinical suspicion should be maintained until all the common causes of oral lesions are ruled out from the differential diagnosis.

International Study Group diagnostic guidelines:

According to the International Study Group guidelines, for a patient to be diagnosed with Behçet’s disease,[3] the patient must have oral (aphthous) ulcers (any shape, size or number at least 3 times in any 12 months period)along with 2 out of the next 4 “hallmark” symptoms:

*genital ulcers (including anal ulcers and spots in the genital region and swollen testicles or epididymitis in men)
*skin lesions (papulo-pustules, folliculitis, erythema nodosum, acne in post-adolescents not on corticosteroids)
*eye inflammation (iritis, uveitis, retinal vasculitis, cells in the vitreous)
*pathergy reaction (papule >2 mm dia. 24-48 hrs or more after needle-prick). The pathery test has a specificity of 95% to 100%, but the results are often negative in American and European patients

Despite the inclusive criteria set forth by the International Study Group, there are cases where not all the criteria can be met and therefore a diagnosis can not readily be made. There is however a set of clinical findings that a physician can rely upon in making a tenative diagnosis of the disease; essentially Behçet’s disease does not always follow the International Study Group guidelines and so a high degree of suspicion for a patient who presents having any number of the following findings, is necessary:

*mouth ulcers
*arthritis/arthralgia
*nervous system symptoms
*stomach and/or bowel inflammation
*deep vein thrombosis
*superficial thrombophlebitis
*cardio-vascular problems of an inflammatory origin
*inflammatory problems in chest and lungs
*problems with hearing and/or balance
*extreme exhaustion
*changes of personality, psychoses
*any other members of the family with a diagnosis of Behçet disease.

Pathogenesis:
The etiology is not well-defined, but it is primarily characterized by auto-inflammation of the blood vessels. Although sometimes erroneously referred to as a “diagnosis of exclusion,” the diagnosis can sometimes be reached by pathologic examination of the affected areas.

The primary mechanism of the damage is an overactive immune system that seems to target the patient’s own body. The primary cause is not well known. In fact, as of now, no one knows why the immune system starts to behave this way in Behçet’s disease. There does however seem to be a genetic component involved as first degree relatives of the affected patients are often affected in more than expected proportion for the general population.

Treatment:
There’s no cure for Behcet’s yet, but research continues and treatment is available to keep inflammation and symptoms at bay.

Current treatment is aimed at easing the symptoms, reducing inflammation, and controlling the immune system. High dose Corticosteroid therapy (1 mg/kg/d oral prednisone) is indicated for severe disease manifestations. Anti-TNF therapy such as infliximab has shown promise in treating the uveitis associated with the disease. Another Anti-TNF agent, Etanercept, may be useful in patients with mainly skin and mucosal symptoms.

Interferon alfa-2a may also be an effective alternative treatment, particularly for the genital and oral ulcers as well as ocular lesions. Azathioprine, when used in combination with interferon alfa-2b also shows promise, and Colchicine can be useful for treating some genital ulcers, erythema nodosum, and arthritis.

Thalidomide has also been used due to its immune-modifying effect. Dapsone and rebamipide have been shown, in small studies, to have beneficial results for mucocutaneous lesions.

Rest and Exercise:
Although rest is important during flares, doctors usually recommend moderate exercise, such as swimming or walking, when the symptoms have improved or disappeared. Exercise can help people with Behçet’s disease keep their joints strong and flexible.

Pathophysiology
HLA-B51 is strongly associated with Behçet’s disease Behçet disease is considered more prevalent in the areas surrounding the old silk trading routes in the Middle East and in Central Asia. Thus, it is sometimes known as Silk Road Disease. However, this disease is not restricted to people from these regions. A large number of serological studies show a linkage between the disease and HLA-B51. HLA-B51 is more frequently found from the Middle East to South Eastern Siberia, but the incidence of B51 in some studies was 3 fold higher than the normal population. However, B51 tends not to be found in disease when a certain SUMO4 gene variant is involved, and symptoms appear to be milder when HLA-B27 is present. At the current time, a similar infectious origin has not yet been confirmed that leads to Behçet’s disease, but certain strains of Streptococcus sanguinis has been found to have a homologous antigenicity.

Prognosis:
Most people with Behçet’s disease can lead productive lives and control symptoms with proper medicine, rest, and exercise. Doctors can use many medicines to relieve pain, treat symptoms, and prevent complications. When treatment is effective, flares usually become less frequent. Many patients eventually enter a period of remission (a disappearance of symptoms). In some people, treatment does not relieve symptoms, and gradually more serious symptoms such as eye disease may occur. Serious symptoms may appear months or years after the first signs of Behçet’s disease.

Risk Factors
A risk factor is something that increases your chances of getting a disease or condition. Although the exact cause of Behcet’s disease is unknown, some groups of people are more likely to develop the condition than others. Risk factors include:

*Location: the Middle East, Asia, and Japan
*Sex:
*In the US, men are more likely than women to develop this condition.
*In the Middle East, Asia, and Japan, women are more likely than men to develop Behcet’s.
*Age: 20s and 30s

Researches:
Researchers are exploring possible genetic, bacterial, and viral causes of Behçet’s disease as well as improved drug treatment. For example, genetic studies show strong association of the gene HLA-B51 with the disease, but the exact role of this gene in the development of Behçet’s is uncertain. Researchers hope to identify genes that increase a person’s risk for developing Behçet’s disease. Studies of these genes and how they work may provide new understanding of the disease and possibly new treatments.

Researchers are also investigating factors in the environment, such as bacteria or viruses, that may trigger Behçet’s disease. They are particularly interested in whether Streptococcus, the bacterium that causes strep throat, is associated with Behçet’s disease. Many people with Behçet’s disease have had several strep infections. In addition, researchers suspect that herpesvirus type 1, a virus that causes cold sores, may be associated with Behçet’s disease.

Finally, researchers are identifying other medicines to better treat Behçet’s disease. TNF inhibitors are a class of drugs that reduce joint inflammation by blocking the action of a substance called tumor necrosis factor (TNF). Although serious side effects have been reported for TNF inhibitors, they have shown some promise in treating Behçet’s disease. Examples of TNF inhibitors include etanercept and infliximab. TNF inhibitors belong to a family of drugs called biologics, which target the immune response. Also, interferon alpha, a protein that helps fight infection, has shown promise in treating Behçet’s disease. Thalidomide, which is believed to be a TNF inhibitor, appears effective in treating severe mouth sores, but its use is experimental and side effects are a concern. Thalidomide is not used to treat women of childbearing age because it causes severe birth defects.

Where Can People Get More Information About Behçet’s Disease?

•National Institute of Arthritis and Musculoskeletal and Skin Diseases (NIAMS)
Information Clearinghouse
National Institutes of Health

1 AMS Circle
Bethesda,  MD 20892-3675
Phone: 301-495-4484
Toll Free: 877-22-NIAMS (226-4267)
TTY: 301-565-2966
Fax: 301-718-6366
Email: NIAMSinfo@mail.nih.gov
Website: http://www.niams.nih.gov

•National Institute of Dental and Craniofacial Research (NIDCR)
National Institutes of Health
45 Center Drive, MSC 6400
Building 45, Room 4AS-25
Bethesda,  MD 20892-2510
Phone: 301-496-4261
Email: nidcrinfo@mail.nih.gov
Website: http://www.nidcr.nih.gov

•National Institute of Diabetes and Digestive and Kidney Diseases (NIDDK)

National Institutes of Health
1 Information Way
Bethesda,  MD 20892-3560
Toll Free: 800-860-8747
TTY: 866-569-1162
Fax: 703-738-4929
Email: ndic@info.niddk.nih.gov
Website: http://www.niddk.nih.gov

•National Eye Institute (NEI)
National Institutes of Health
31 Center Drive MSC 2510
Bethesda,  MD 20892-2510
Phone: 301-496-5248
Email: 2020@nei.nih.gov
Website: http://www.nei.nih.gov

•National Institute of Neurological Disorders and Stroke (NINDS)

NIH Neurological Institute
P.O. Box 5801
Bethesda,  MD 20824
Phone: 301–496–5751
Toll Free: 800–352–9424
TTY: 301–468–5981
Website: http://www.ninds.nih.gov

•American Academy of Dermatology (AAD)
P.O. Box 4014
Schaumberg,  IL 60618-4014
Phone: 847-330-0230
Toll Free: 866-503-SKIN (7546)
Fax: 847-240-1859
Website: http://www.aad.org

•American College of Rheumatology (ACR)
2200 Lake Boulevard NE
Atlanta,  GA 30319
Phone: 404-633-3777
Fax: 404-633-1870
Website: http://www.rheumatology.org

•Dermatology Foundation
1560 Sherman Avenue, Suite 870
Evanston,  IL 60201-4808
Phone: 847-328-2256
Fax: 847-328-0509
Email: dfgen@dermatologyfoundation.org
Website: http://www.dermfnd.org

(This organization is “research only.” Contact should be made by U.S. mail or e-mail.)
•American Behçet’s Disease Association
P.O. Box 869
Smithtown,  NY 11787-0869
Phone: 631-656-0537
Toll Free: 800-7-BEHCET (723-4238)
Fax: 480-247-5377
Website: http://www.behcets.com

•American Skin Association (ASA)
346 Park Avenue S., 4th floor
New York,  NY 10010
Phone: 212-889-4858
Toll Free: 800-499-SKIN
Website: http://www.americanskin.org

•Arthritis Foundation
P.O. Box 7669
Atlanta,  GA 30357-0669
Phone: 404-872-7100
Toll Free: 800-283-7800
Website: http://www.arthritis.org

•National Organization For Rare Disorders (NORD)
55 Kenosia Avenue, P.O. Box 1968
Danbury,  CT 06813-1968
Phone: 203-744-0100
Toll Free: 800-999-6673
TTY: 203-797-9590
Fax: 203-798-2291
Email: orphan@rarediseases.org
Website: http://www.rarediseases.org

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/behcets1.shtml
http://www.niams.nih.gov/Health_Info/Behcets_Disease/default.asp#beh_i
http://en.wikipedia.org/wiki/Beh%C3%A7et’s_disease
http://www.lifescript.com/Health/A-Z/Conditions_A-Z/Conditions/B/Behcets_disease.aspx?gclid=CNSczv6IkqcCFcZw5Qodbmg-cw&trans=1&du=1&ef_id=5jhNXmWk-CMAAMAQ:20110218155657:s

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Freedom From the Daily JAB

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Indian scientists are using tissue engineering to give diabetes patients new insulin-making cells……...CLICK & SEE

Biomaterials scientist Prabha Nair is pitting her expertise of polymers to hold out a new line of hope for patients with diabetes who are dependent on insulin shots. In her laboratory, she has used two structures fashioned out of polymer materials to normalise blood sugar in rats with diabetes for up to 90 days. One of the polymer structures is designed to make insulin-secreting cells function properly, while the other is intended to protect such cells from threats that might emerge from the body’s immune system.

Nair and her colleagues at the government-funded Sree Chitra Tirunal Institute of Medical Sciences and Technology (SCTIMST), Thiruvananthapuram have combined two applications of polymers to tackle two major obstacles that have held back a promising but experimental treatment for diabetes from widespread use. The treatment, called islet cell transplantation, involves the removal of insulin-secreting cells from the pancreas of a deceased organ donor and their implantation into a patient with diabetes.

It is nearly a decade since researchers at the University of Alberta in Edmonton, Canada, demonstrated that islet cell transplantation may help patients with diabetes acquire normal blood sugar levels and achieve some level of freedom from the need for insulin.

A review of islet transplantation on 225 patients between 1999 and 2006 had revealed several benefits — including reduced need for insulin, improved blood glucose control, and lowered risk of hypoglycemia, according to the National Institute of Diabetes and Digestive and Kidney Disorders in the US. Two years after the islet transplantation, about one-third of the recipients were free of the need for insulin shots, the review suggested.

Islet cell transplantation, however, is not standard therapy yet. “There is a critical shortage of islet cells because of a shortage of organ donors,” says Nair, a scientist in the division of tissue engineering and regeneration technologies at the SCTIMST.

Patients who receive islet cells need to take immunosuppressive drugs throughout their lives to prevent their immune systems from destroying the implanted cells. These drugs have side effects including an increased risk of cancer.

The SCTIMST researchers harvested a class of cells known as pancreatic progenitor cells from mice and placed them in a cocktail of appropriate biochemicals where they turn into insulin-secreting islet-like cells.

The scientists then loaded these islet-like cells into three-dimensional scaffolds constructed out of a gelatin, a natural polymer, and polyvinylpyrrolidone, a synthetic polymer. The islet-like cells proliferate on the scaffolds and serve as a potential source of insulin.

In experiments, the scientists observed that rats with diabetes that received these islet cell-bearing scaffolds alone died within 20 days. Their scaffold cells had been attacked by the rats’ immune systems, leading to the destruction of tissue and the failure of the implantation.

“We also designed a polymer capsule to shield the implanted islet cells from the immune system,” Nair told KnowHow. When the scientists combined the scaffolding, also called tissue engineering, with encapsulation, the rats survived for up to 90 days.

The rats were models for type-I, or insulin-dependent diabetes, but researchers say the tissue engineering and encapsulation strategy may also be considered as a possible option for patients with adult-onset diabetes who need insulin injections. Given the differences in the lifespans of rats and humans, some researchers believe the 90-day freedom from insulin observed in the laboratory animals may be equivalent to several years in humans — although exactly how long is still a subject of debate.

“These results are really exciting,” says Aroop Dutta, a tissue engineering specialist and founder of ExCel Matrix Biologicals, a Hyderabad-based start-up in biomaterials and tissue engineering, who was not connected with the research in Thiruvananthapuram.

“There just aren’t enough human-derived islet cells for the large numbers of diabetes patients dependent on insulin. Animal cells or stem cell-based approaches are the only viable options as sustained sources of islet cells,” he adds.

The results of the SCTIMST’s experiments were published last Friday in the journal Acta Biomaterialia. The researchers say their use of islet cells from mice in rats with diabetes suggests that the polymer capsule that keeps the immune system at bay may facilitate xenotransplants — the use of cells or organs across species — as an option for reversing diabetes. “But there is still much work to be done,” Nair cautions.

“We’ll need to establish that this also works in large animals,” she said. The SCTIMST group plans to initiate studies in pigs with diabetes. If the technique is indeed shown to work in large animals too, it could be ready for human clinical trials within two or three years.

Source : The Telegraph ( kolkata, India)

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Phthalates Cause Inflammation in At-Risk Babies

Researchers have identified a direct link between substances that make plastics more pliable, and inflammation in newborns. They are encouraging limiting the use of the plasticizers.
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Premature babies are exposed to extraordinarily high concentrations of phthalates due to exposure to plastic medical equipment used during neonatal intensive care.

Many of the diseases unique to premature babies, including the lung disorder bronchopulmonary dysplasia and the intestinal ailment necrotizing enterocolitis, are associated with excessive inflammation.

Newswise reports:

“… [There is] direct evidence that the presence of phthalates prolongs the survival of white blood cells, which supports the idea that they are contributing to damage and to inflammation … phthalates encourage cells to produce hydrogen peroxide, which … can kill cells and damage tissues.


You may click to see :-

Health Risks of Phthalates

Resources:
Newswise July 20, 2010
Pediatric Research August 2010; 68(2):134-9

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Ailmemts & Remedies

Skin Colour

Indians are very conscious of their skin colour. There is great alarm and anxiety if the skin suddenly develops white patches. About 1 per cent of the population is affected by this condition — called leukoderma (white skin) or vitiligo (“streaked calf” in Greek). The patches usually appear between the ages of 12 and 40. The disease affects people in all socio-economic strata. Michael Jackson was affected by it. Other rich and famous sufferers are Amitabh Bachchan and Gautam Singhania, the chairman and managing director of Raymonds.
……..click to see the picture
The sudden loss of pigmentation causes 25 per cent of these people to become obsessed with their skin colour, depressed or even suicidal. Money does not make the disease disappear; it only makes it possible to consult the world’s best dermatologists.

The de-pigmentation often starts on the hands and feet. In the case of Jackson, it first appeared on his hands. This was the reason behind his signature white glove. In others it may start around orifices like the nose, mouth, eyes, umbilicus, genital areas and rectum. The patches may remain stationary, increase in size or spread over the whole body. They are symmetrical on both sides of the body. Some areas may suddenly re-pigment while the white patches continue to spread.

The loss of colour is due to the mutation of one of the genes on chromosome 17. This is usually inherited. The mutations may remain unexpressed and the person may be normal all through life. However, if a family member is affected, the risk of vitiligo developing eventually in another member is increased five-fold. The same gene is responsible for premature greying. Some members may have patches, others may develop grey hair in their twenties while still others may appear perfectly normal. The gene may start to express itself and cause de-pigmentation as a result of a trigger like a stressful event. It may also be precipitated by an injury or constant friction in shoes or clothing.

The mutated gene triggers an autoimmune disorder and the body forms antibodies against melanocytes (pigment producing cells). The latter are thus destroyed. Vitiligo may be associated with other autoimmune disorders which affect organs such as the thyroid, stomach and adrenal glands. It may form part of the spectrum of systemic lupus (an autoimmune disease that affects all the organs in the body, and is thus difficult to diagnose).

Sometimes a white baby is born to a “normal” family. The entire skin, hair and even the eyes lack pigment. This condition is called albinism and the person is referred to as an albino. It occurs because the melanocytes are unable to produce melanin, the colouring pigment. This is also an inherited condition but since the gene is recessive it does not express itself and manifest itself as a “white baby” unless it is inherited from both parents. A person who carries the gene may look normal and not be aware of it. If he or she incidentally marries another carrier, the child can be albino.

The pigment producing melanocytes may be absent from birth in certain areas. This hereditary condition is called piebaldism. It can occur anywhere, and can result in just a white forelock — like in the case of Indira Gandhi.

Owing to the similarity in symptoms, vitiligo is sometimes confused with piebaldism, albinsim or even leprosy. White scars may give rise to a similar appearance. A diagnosis can be reached by a skin biopsy.

It is better to avoid sunlight when vitiligo first appears. As the skin tans, the areas without melanin become obvious. Use an umbrella or apply SF (sunfilter) 30 sunscreen on the exposed areas.

Small patches can be camouflaged with cosmetics. They can also be treated under supervision by applying steroid creams. Constant use of these creams, however, can damage the skin texture.

Physicians in India and Egypt documented vitiligo as early as 1,500 BC. They treated it by applying and administering extracts of the fruit, seeds and leaves of two plants — Psoralea coryifolia Linnaues and Ammi majus Linnaeus. Even today, isolates of these plants are successfully used topically and orally. Synthetic compounds are also available. They act by increasing sensitivity to light and augmenting pigmentation in the affected areas (photochemotherapy). Treatment usually involves exposure to a measured amount of natural sunlight (PUVASOL) or artificial UV radiation (PUVA) to induce re-pigmentation. Phototherapy (without light-sensitising chemicals) can also be used. Sunburn is a common complication.

Surgical treatment can be tried by using skin grafts from pigmented areas. The success rate varies between 65 and 90 per cent. If the de-pigmented areas are extensive, some patients bleach the remaining dark portions of the skin to achieve a universal white colour.

Source: The Telegraph ( Kolkata, India)

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