Amyloid heart disease


Amyloid Heart Disease

Description, Causes and Risk Factors:

Also called by the name amyloidosis.

Antibodies, or immunoglobulins, are important agents in the body's immune system. An antibody is capable of identifying antigens such as bacteria and viruses. An antibody is composed of four strings of amino acids called polypeptides. Two of these are "heavy" chains, and two are "light." The four amino acid chains combine to form a Y-shaped antibody molecule. Amyloid is a starchlike substance. A hard waxy deposit consisting of protein and polysaccharides that results from the degeneration of tissue.

Amyloid Heart Disease is a group of diseases in which abnormal proteins, called amyloid proteins, accumulate in organs or organ systems such as the heart, kidneys, nervous system or gastrointestinal tract. There are different types of amyloidosis, dependent on the type of protein being deposited in tissues.

Terminology: Primary cardiac amyloidosis, secondary cardiac amyloidosis, senile amyloidosis, amyloidosis cutis, amyloidosis of aging, amyloidosis of multiple myeloma, chronic amyloidosis, familial amyloidosis, focal amyloidosis, hereditary amyloidosis, lichen amyloidosis, lichenoid amyloidosis, light chain-related amyloidosis, macular amyloidosis, nodular amyloidosis, primary amyloidosis, renal amyloidosis, secondary amyloidosis, senile amyloidosis.

Amyloidosis can affect any organ including brain, thyroid, heart, kidneys, liver, lymph, adrenal glands, nodes, muscle tissue, nerves, pancreas, spleen.

Types of Amyloidosis:

  • Primary amyloidosis - Begins in the bone marrow and primarily affects heart, kidneys, tongue, nerves and intestines. Not usually associated with other conditions.

  • Secondary amyloidosis - Associated with chronic infectious or inflammatory diseases. Mostly affects kidneys, spleen, liver and lymph nodes. Other organs may be affected.

  • Familial amyloidosis - Inherited condition which affects nerves, heart, and kidneys.

In primary amyloidosis (AL) the fibrils consist of the variable portions of monoclonal kappa (?), lambda (?) immunoglobulin light chains or, very rarely, heavy chains. In secondary amyloidosis (AA) the fibrils consist of protein A, a nonimmunoglobulin. In familial amyloidosis (AF) the fibrils are composed of mutant transthyretin (prealbumin) or, rarely, fibrinogen or apolipoprotein.

The cause of Amyloid Heart Disease is not exactly known. It has however been observed that the patients suffering from amyloidosis are aged above 40. To make matters more complex, Amyloid Heart Disease is not a single disease, and there are many different types of amyloid proteins that can be involved. For example, Alzheimer's disease and Creutzfeldt-Jakob disease are two distinct conditions characterized by amyloid deposits in the brain, but the proteins involved are different.

Some studies have related the cause of amyloidosis to the diet consumed by the patients. Treatment hence is given to patients suffering from amyloidosis by making a relative change in the diet. Amyloid Heart Disease has not limited itself to any particular sex or region, culture or creed of people.

Amyloid heart disease

Cardiac amyloidosis usually occurs during primary amyloidosis. Primary amyloidosis is often seen in people with multiple myeloma cancer. Cardiac amyloidosis occurs when amyloid deposits take the place of normal heart muscle. It is the most typical type of restrictive cardiomyopathy. Cardiac amyloidosis may affect the way electrical signals move through the heart. This can lead to arrhythmias and conduction disturbances.

Symptoms:

Symptoms include:

  • Excessive urination at night.

  • Fatigue.

  • Reduced activity tolerance.

  • Palpitations.

  • Shortness of breath with activity.

  • Swelling of legs, ankles, or other part of the body.

  • Trouble breathing while lying down.

  • Fatigue.

  • Weight loss.

  • Numbness or tingling in the hands or feet.

  • Diarrhea.

  • An enlarged tongue.

  • Feeling full after eating smaller amounts of food than usual.

  • Dizziness upon standing.

  • Fainting spells.

Diagnosis:

A complete medical history and physical examination is essential for diagnosing Amyloid Heart Disease. Blood or urine tests can detect the amyloid proteins, but only bone marrow tests (biopsy of the small sample of tissue) can positively establish the diagnosis of amyloidosis. A small tissue sample, or biopsy, will often be taken from the abdominal fat, rectum, and/or bone marrow to confirm the diagnosis of amyloidosis. These biopsies are relatively minor procedures done in an outpatient clinic with a local anesthetic (numbing medication). Occasionally, samples need to be taken from damaged organs such as the liver, intestines, heart or kidney. The type of biopsy procedure required will vary from patient to patient.

Treatment:

As Amyloid Heart Disease is not a particular disease, there is no specific treatment for the same. What needs to be done is that, the production of the protein amyloid needs to be controlled; hence medication for the same can be prescribed, but there is no specific medication that has proved that it controls the production of amyloid by the bone marrow. Doctors also advise the patients to go in for a diet change, to see if this controls the production of the amyloid.

Some patients with primary amyloidosis respond to chemotherapy directed at the abnormal plasma cells. Stem cell transplantation may be used, as in multiple myeloma. 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.

A number of treatment modalities including special diets, vitamins, DMSO (dimethyl sulfoxide) have been tried without any positive results. In some patients, treatment with high doses intravenous melphalan followed by bone marrow stem cell rescue (autologous bone marrow transplant) has proven effective. This therapy however is not indicated for patients with severe heart amyloidosis or extensive disease in other organs. The most common treatment for primary amyloidosis that is used by physicians includes chemotherapy consisting of Alkeran (Melphalan) and Prednisone. These drugs have been shown to be effective in the treatment of multiple myeloma which is also a disorder of the immune system but which is generally considered to be a malignant disease. Some studies have suggested that chronic treatment with Colchicine, which has been used for many years for the treatment of gout, may slow down the progress of primary amyloidosis.

Although no link has been established between diet and development of amyloid proteins, a patient whose heart or kidneys have been affected by the disease may be advised to use a diuretic or follow a low-salt diet.

Disclaimer: The following tests, drugs and medications, surgical procedures are in some way related to, or used in the treatment. The information provided herein should not be used during any medical emergency or for the diagnosis or treatment of any medical condition.

DISCLAIMER: This information should not substitute for seeking responsible, professional medical care.

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