answersLogoWhite

0

Definition

Hereditary 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 Names

Amyloidosis - hereditary

Causes, incidence, and risk factors

Hereditary 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 cause
  • Secondary, which means it results from diseases such as cancer of the blood cells (myeloma)

For further information, see the specific type:

References

Gertz 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.

User Avatar

Wiki User

15y ago

What else can I help you with?

Related Questions

What are the major forms of amyloidosis?

The major forms of amyloidosis include primary amyloidosis (AL), secondary amyloidosis (AA), and hereditary amyloidosis (ATTR). AL amyloidosis is caused by the abnormal production of immunoglobulin light chains, AA amyloidosis is caused by chronic inflammation leading to the deposition of serum amyloid A protein, and ATTR amyloidosis is caused by mutations in the transthyretin gene. Each form of amyloidosis has distinct clinical manifestations and treatment approaches.


What form of CAA is Flemish type of hereditary cerebral hemorrhage with amyloidosis?

autosomal dominant, with a mutation involving the amyloid precursor protein. Symptoms include brain hemorrhage or dementia.


Is syringomyelia hereditary?

Syphilis is not hereditary.


What is a hereditary infection?

Is is an infection that is hereditary


Is aids hereditary?

No it isn't hereditary, it either has to be through a shag or blood transfusion.AIDS is not hereditary.


How do you write a sentence using the word hereditary?

I can give you several sentences.That is a hereditary trait.He has a hereditary illness.Her family's hereditary home is in London.


Is the disease leucoderma hereditary?

no albinism is a hereditary.


Is Sacrococcygeal teratoma hereditary or environmental?

hereditary


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.


Is Orthopnea hereditary?

Orthopnea is not hereditary. It is a symptom of a disease.


How can you use hereditary in a sentence?

There are plenty of ways to use hereditary in a sentence. You could say that your brown hair is hereditary.


Where the hereditary material of the cell is hereditary In bacteria?

With the exception of bacteria cells, hereditary material is held in the cell's nucleus.