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Primary amyloidosis is a disorder in which protein fibers are deposited in tissues and organs, harming them.
Alternative NamesAmyloid - primary
Causes, incidence, and risk factorsThe 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- 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
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 biopsycan 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.
- Urinalysisshows 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
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.
Expectations (prognosis)The severity of the disease depends upon the organs affected. Heart and kidney involvement may lead to organ failure and death. Systemicinvolvement is associated with death within 1 to 3 years.
Complications- Congestive heart failure
- Death
- Endocrinefailure (hormonal disorder)
- Kidney failure
- Respiratoryfailure
Call your health care provider if symptoms consistent with primary amyloidosis develop.
If you know you have primary amyloidosis, call your health care provider if difficulty breathing, persistent swelling of the ankles or other areas, decreased urine output, or other symptoms occur. This may indicate that complications have developed.
PreventionThere is no known prevention.
ReferencesBuxbaum JN. The Amyloidoses. In: Goldman L, Ausiello D. Cecil Textbook of Medicine. 22nd ed. Philadelphia, Pa: WB Saunders; 2004.
Hoffman R, Benz Jr. EJ, Shattil SJ, et al., eds. Hematology: Basic Principles and Practice. 4th ed. Philladelphia, Pa: Churchill Livingston; 2005:1540-48.
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∙ 13y ago
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∙ 12y agoPrimary amyloidosis is a disorder in which abnormal proteins build up in tissues and organs. Clumps of the abnormal proteins are called amyloid deposits.
Alternative NamesAmyloid - primary
Causes, incidence, and risk factorsThe cause of primary amyloidosis is unknown. The condition is related to abnormal and excess production of antibodies by a type of immune cell called plasma cells.
Primary amyloidosis can lead to conditions that include:
- Carpal tunnel syndrome
- Heart muscle damage (cardiomyopathy) leading to congestive heart failure
- Intestinal malabsorption
- Liver enlargement
- Kidney failure
- Nephrotic syndrome
- Neuropathy (nerves that do not work properly)
- Orthostatic hypotension (abnormal drop in blood pressure with standing)
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.
SymptomsSymptoms 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.
Symptoms include:
- Abnormal heart rhythm
- Enlarged tongue
- Fatigue
- Numbness of hands and feet
- Shortness of breath
- Skin changes
- Swallowing problems
- Swelling in the arms and legs
- Weak hand grip
- Weight loss
Other symptoms that may occur with this disease:
- Decreased urine output
- Diarrhea
- Hoarseness or changing voice
- Joint pain
- Weakness
Your doctor may discover that you have an enlarged liver or spleen.
If 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 aspiration, rectal mucosa biopsy, or 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.
- Urinalysisshows protein, casts, or fat bodies.
This disease may also affect 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
Some patients with primary amyloidosis respond to chemotherapy focused on the abnormal plasma cells. A stem cell transplant may be done, as in multiple myeloma.
In secondary amyloidosis, aggressively treating the disease that is causing the excess amyloid protein can improve symptoms and/or slow the disease from getting worse. Complications such as heart failure, kidney failure, and other problems can sometimes be treated, when needed.
Expectations (prognosis)How severe the disease is depends on the organs that are affected. When the heart and kidney are involved, it may lead to organ failure and death. Body-wide ( systemic) involvement can lead to death in 1 to 3 years.
Complications- Congestive heart failure
- Death
- Endocrinefailure (hormonal disorder)
- Kidney failure
- Respiratoryfailure
Call your health care provider if:
- You have symptoms of primary amyloidosis
- You know you have primary amyloidosis and you have difficulty breathing, persistent swelling of the ankles or other areas, decreased urine output, or other symptoms that may indicate complications have developed
There is no known prevention for primary amyloidosis.
ReferencesBuxbaum JN. The Amyloidoses. In: Goldman L, Ausiello D. Cecil Textbook of Medicine. 22nd ed. Philadelphia, Pa: WB Saunders; 2004.
Gertz MA, Lacy MQ, Dispenzieri A, Hayman SR. Immunoglobulin light-chain amyloidosis (primary amyloidosis). In: Hoffman R, Benz Jr. EJ, Shattil SJ, et al., eds. Hematology: Basic Principles and Practice. 5th ed. Philladelphia, Pa: Elsevier Churchill Livingston; 2008:chap 89.
Reviewed ByReview Date: 02/20/2011
David C. Dugdale, III, MD, Professor of Medicine, Division of General Medicine, Department of Medicine, University of Washington School of Medicine. Also reviewed by David Zieve, MD, MHA, Medical Director, A.D.A.M., Inc.
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What are the major forms of amyloidosis?
The major forms of this disease are primary systemic, secondary, and familial or hereditary amyloidosis. There is also another form of amyloidosis associated with Alzheimer's disease.
Hereditary amyloidosis?
DefinitionHereditary amyloidosis is a condition in which abnormal protein deposits (called amyloid) form in almost every tissue in the body. These protein deposits damage the tissues and interfere with the function of the involved organs.Alternative NamesAmyloidosis - hereditaryCauses, incidence, and risk factorsHereditary amyloidosis is passed down from parents to their children (inherited). For more information see: Primary amyloidosis.Other types of amyloidosis, which are not inherited, may be:Spontaneous, which means it occurs without a known causeSecondary, which means it results from diseases such as cancer of the blood cells (myeloma)For further information, see the specific type:Cardiac amyloidosisCerebral amyloidosisSecondary systemic amyloidosisReferencesGertz MA, Lacy MQ, Dispenzieri A, Hayman SR. Immunoglobin light-chain amyloidosis (primary amyloidosis). In: Hoffman R, Benz EJ Jr., Shattil SS, et al., eds. Hematology: Basic Principles and Practice. 5th ed. Philadelphia, Pa: Churchill Livingston Elsevier; 2008: chap 89.
Cardiac amyloidosis?
DefinitionCardiac amyloidosis is a disorder caused by deposits of an abnormal protein (amyloid) in the heart tissue, which make it hard for the heart to work properly.Alternative NamesAmyloidosis - cardiac; Primary cardiac amyloidosis - AL type; Secondary cardiac amyloidosis - AA type; Stiff heart syndrome; Senile amyloidosisCauses, incidence, and risk factorsAmyloidosisrefers to a family of diseases in which there is a buildup of clumps of proteins called amyloids in body tissues and organs. These proteins slowly replace normal tissue, leading to failure of the involved organ. There are many forms of amyloidosis.Cardiac amyloidosis usually occurs during primary amyloidosis (AL type amyloidosis). Primary amyloidosis is often seen in people with multiple myeloma cancer.Cardiac amyloidosis ("stiff heart syndrome") 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 (conduction system). This can lead to arrhythmias and conduction disturbances (heart block).Secondary amyloidosis (AA type) rarely affects the heart. However, a form of secondary amyloidosis called senile amyloidosis involves the heart and blood vessels. Senile amyloidosis is caused by overproduction of a different protein. The condition is becoming more common as the average age of the population increases.Cardiac amyloidosis is more common in men than in women. The disease is rare in people under age 40.SymptomsExcessive urination at nightFatigue, reduced activity tolerancePalpitations(sensation of feeling heart beat)Shortness of breath with activitySwelling of legs, ankles, or other part of the body (See: abdominal swelling)Trouble breathing while lying downSome patients may have no symptoms.Signs and testsIt can be difficult to diagnose cardiac amyloidosis, because the signs can be related to a number of different conditions.Signs may include:Abnormal sounds in the lung (lung crackles) or a heart murmurBlood pressurethat is low or drops when you stand upEnlarged neck veinsSwollen liverThe following tests may be performed to help diagnose cardiac amyloidosis:Chest or abdomen CT scan (this is considered the "gold standard")Coronary angiographyEchocardiogramMagnetic resonance imaging (MRI)Nuclear heart scans (MUGA, RNV)An ECG may show problems with the heart beat or heart signals (conduction disturbance).A cardiac biopsyis used to confirm the diagnosis. A biopsy of another area, such as the abdomen, kidney, or bone marrow, is often done to confirm the diagnosis.TreatmentYour doctor may tell you to change your diet. This may include salt and fluid restrictions.You may need to take water pills (diuretics) to help your body remove excess fluid. The doctor may tell you to weigh yourself every day. A weight gain of 3 or more pounds over 1 - 2 days can mean there is too much fluid in the body.Digoxin, calcium channel blockers, and beta blockers may be used with caution in patients with atrial fibrillation. However, the dosage must be carefully monitored. Patients with cardiac amyloidosis may be unusually sensitive to any side effects.Other treatments may include:ChemotherapyImplantable cardioverter-defibrillator (AICD)Pacemaker, if there are problems with heart signalsPrednisone, an anti-inflammatory medicineA heart transplant may be considered for some patients with very poor heart function. However, it is not done in those with AL type amyloidosis, because the disease weakens many other organs. People with hereditary amyloidosis will need a liver transplant.Expectations (prognosis)Cardiac amyloidosis is a long-term (chronic) condition that slowly gets worse. On average, persons with cardiac amyloidosis live less than 1 year.ComplicationsAtrial fibrillation or ventricular arrhythmiasCongestive heart failureFluid buildup in the abdomen (ascites)Increased sensitivity to digoxinLow blood pressure and dizziness from excessive urination (due to medication)Sick sinus syndromeSymptomaticcardiac conduction system disease (arrhythmias related to abnormal conduction of impulses through the heart muscle)Calling your health care providerCall your health care provider if you have this disorder and new symptoms develop, particularly:Dizziness when you change positionExcessive weight (fluid) gainExcessive weight lossFaintingspellsSevere breathing difficultyReferencesHare JM. The Dilated, Restrictive, and Infiltrative Cardiomyopathies. In: Libby P, Bonow RO, Mann DL, Zipes DP. Braunwald's Heart Disease: A Textbook of CardiovascularMedicine. 8th ed. Philadelphia, Pa: Saunders Elsevier; 2007:chap 64.
What are some phrases with the word primary in them?
primary colors primary election primary source of income primary residence or primary place of residence primary function primary caregiver
Secondary systemic amyloidosis?
DefinitionSecondary systemic amyloidosis is a disorder in which abnormal proteins build up in tissues and organs. Clumps of the abnormal proteins are called amyloid deposits.Secondary means it occurs because of another disease or situation. For example, this condition usually occurs as a result of chronicinfection or chronic inflammatory disease.Systemic means body-wide. For example, a systemic disease affects the entire body.See also:Cardiac amyloidosisHereditary amyloidosisPrimary amyloidosisAlternative NamesAmyloidosis - secondary systemicCauses, incidence, and risk factorsThe exact cause of amyloidosis is unknown. You are more likely to develop secondary systemic amyloidosis if you have a long-term infection or inflammation.This condition may occur along with:Ankylosing spondylitisBronchiectasisChronic osteomyelitisCystic fibrosisHairy cell leukemiaHodgkin's diseaseLong-term skin abscess and certain ulcersLong-term kidney dialysisJuvenile chronic arthritisMultiple myelomaReiter syndromeRheumatoid arthritisSjogren syndromeSystemic lupus erythematosusTuberculosisSymptomsBleeding in the skinFatigueIrregular heartbeatNumbness of hands and feetShortness of breathSwallowing difficultiesSwollen arms or legsSwollen tongueWeak hand gripWeight lossSigns and testsTests that may be done include:Abdominal ultrasound (may show a swollen liver or spleen)Biopsy or aspiration of fat just beneath the skin (subcutaneous fat)Biopsy of rectumBiopsy of skinBiopsy of bone marrowBlood tests, including creatinine and BUNECGEchocardiogramNerve conduction velocityUrinalysisTreatmentThe condition that is causing the amyloidosis should be treated. Some persons may need a bone marrow or stem cell transplant. There is no specific treatment for amyloidosis itself.Expectations (prognosis)How well a person does depends on which organs are affected. If the disease involves the heart and kidneys, it may lead to organ failure and death.ComplicationsEndocrinefailureHeart failureKidney failureRespiratoryfailureCalling your health care providerCall your health care provider if you have symptoms of this condition. The following are serious symptoms that require prompt medical attention:BleedingIrregular heartbeatsNumbnessShortness of breathSwellingWeak gripPreventionIf you have a disease that's known to increase your risk of this condition, make sure you get it treated. This may help prevent amyloidosis.ReferencesBuxbaum JN. The amyloidoses. In: Goldman L, Ausiello D, eds. Cecil Medicine. 23rd ed. Philadelphia, Pa: Saunders Elsevier; 2007: chap 296.
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Isolation of a child with measles belongs to what level of prevention?
Secondary
Is a picture a primary or secondary source?
A picture can indeed be a primary source.
Is Grade 3 is primary or pre-primary?
primary
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All saints primary ,Elliott Belgrave primary,Roland rewards primary,st.Lucy primary, and st .George lions
Is a diary primary or secondary?
It is a primary source.
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Primary- it comes from someone who was a witness to a historical event (In this case, the Holocaust).
