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Amyloidosis in Iran Details

What is Amyloidosis?

A cardiovascular disease where there is an unusual accumulation of proteins in different body organs is known as Amyloidosis.

The amyloid proteins can grow in different places that include gut, kidneys, heart, and nerves.

This is considered a serious condition that may cause life-threatening organ failure.

When amyloid is deposited into the kidney, then kidneys are unable to filter out toxins and waste from the blood then it is known as renal (kidney) Amyloidosis. Amyloidosis is responsible for affecting the kidneys that may result in following conditions that include:

The high protein level in the urine
Indications of kidney failure that include swelling in the ankles and feet and puffiness surrounding the eyes

Types of Amyloidosis

AL Amyloidosis(Primary amyloidosis)

AL Amyloidosis is considered as the most common type.

Light (L) chains are the name given to amyloid proteins that build up in the tissues.

This type is usually not associated with any other condition, but in some cases, it is connected with multiple myeloma.

AA Amyloidosis(Secondary amyloidosis)

In this type, amyloid protein builds up in the tissues and is termed as A protein.

AA Amyloidosis is connected with a large number of serious diseases that include rheumatoid arthritis, diabetes, inflammatory bowel disease, and tuberculosis.

The liver, lymph nodes, spleen, kidneys, and adrenal glands can also be involved.

Hereditary Amyloidosis (ATTR)(Familial amyloidosis)

This is a particular and rare type of Amyloidosis that can be seen in a family coming down from generation to generation.

Hereditary Amyloidosis is responsible for causing eye abnormalities, motor neuropathy problems, carpal tunnel syndrome, and peripheral sensory problems.

Transthyretin (TTR) protein is considered as the most common subtype of hereditary Amyloidosis.

Causes of Amyloidosis

The changes in protein cause Amyloidosis, which make proteins insoluble and also results in the deposition of these proteins in tissues and organs.

Usually, these proteins build up in the tissue space between the cells.

The beginning of Amyloidosis is from the bone marrow.

White and red blood, antibodies, and platelets are produced by the bone marrow that is responsible for protecting the body against many infections.

The body breaks these antibodies when their work is finished.

Amyloidosis develops when the antibodies produced by the bone marrow cells cannot be broken down.

This causes accumulation of antibodies in the blood that gradually deposits in the tissues as amyloid.

The chances of Amyloidosis condition is higher in the following people:

Have a kidney disease where dialysis is required for more than five years
Are more than 50 years of age
Have a family history of Amyloidosis
Have multiple myeloma
Have an inflammatory disease or chronic infection

Symptoms of Amyloidosis

The symptoms depend on the affected organ. It has also been seen that with a such several symptoms, the diagnosis of the Amyloidosis becomes difficult. Some of the common symptoms are:

Palpitations of the heart
Weakness
Fainting spells
Diarrhea
Weight loss
Swelling of the legs or ankles
An enlarged tongue
Shortness of breath
Dizziness upon standing
Severe fatigue
Tingling or numbness in the feet or hands
Feeling full after eating a small quantity of food

Amyloidosis can also result in specific groups of symptoms that are known as syndromes that include:

Malabsorption (problems in absorbing nutrients from food)
Congestive heart failure (inability of the heart to pump sufficient blood to meet the need of a body)
Nerve damage or autonomic neuropathy that may affect the involuntary functions of the body that include perspiration and heart rate.
Nerve damage or peripheral neuropathy may affect the peripheral nervous system (PNS).
The peripheral nervous system is responsible for relaying information between the spinal cord and the brain and the rest of the body.
Nephrotic syndrome is the condition where there is over-accumulation of protein in the kidneys

Diagnosis of Amyloidosis

Physical examination and complete medical history of a patient is thoroughly diagnosed for Amyloidosis.

Bone marrow, blood, and urine tests are also performed.

Urine and blood tests help in detecting the amyloid proteins.

Small samples of tissues (known as biopsies) and bone marrow tests are required for positively stabling the diagnosis of Amyloidosis.

To confirm the diagnosis of Amyloidosis, biopsy, or small tissue samples are taken from the bone marrow, abdominal fat, and rectum.

A specific type of biopsy procedure depends on patient to patient.

Treatment of Amyloidosis

The condition of Amyloidosis has no cure.

A doctor will recommend some treatments for suppressing the development of the amyloid-forming protein and also for managing the symptoms in a patient.

If it found that Amyloidosis is associated with some other condition, then the focus of the treatment is to target the underlying condition.

The specific treatment depends on which organs are affected and the type of Amyloidosis.

Heart or kidney transplant can be suggested
Stem cell transplant helps in removing the substance that causes amyloid formation in those who have primary AL Amyloidosis and who also possess two main damaged organs
Liver transplant helps in stopping the disease in those who are suffering from hereditary Amyloidosis
Patients who have primary AL Amyloidosis are treated with the help of chemotherapy medicines
Steroids that are powerful inflammatory medicines help in fighting off inflammation and are also useful in treating secondary AA Amyloidosis

Some of the other treatments that can help with symptoms include the followings:

Diet modifications for those who have gastrointestinal Amyloidosis
Thickeners are added to the fluids for preventing choking for those who have tongue swelling
Diuretic medicines are used for removing extra water out from the body
Compression stockings are also used for relieving feet or legs swelling