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Hereditary amyloidosis

Definition

Hereditary amyloidosis is a condition in which abnormal protein deposits (called amyloid) form in almost every tissue in the body. Harmful deposits most often form in the heart, kidneys, and nervous system. These protein deposits damage the tissues and interfere with how organs work.

Alternative Names

Amyloidosis - hereditary; Familial amyloidosis

Causes

Hereditary amyloidosis is passed down from parents to their children (inherited). Genes may also play a role in primary amyloidosis.

Other types of amyloidosis are not inherited. They include:

  • Senile systemic: seen in people older than 70
  • Spontaneous: occurs without a known cause
  • Secondary: results from diseases such as cancer of the blood cells (myeloma)

Specific conditions include:

Treatment

Treatment to improve the function of damaged organs will help relieve some symptoms of hereditary amyloidosis. A liver transplant may be helpful to reduce the creation of harmful amyloid proteins. Talk to your health care provider about treatments.

Gallery

Amyloidosis of the fingers
Amyloidosis refers to the extracellular deposition of a protein called amyloid. This protein deposition can affect multiple organs. The deposition of amyloid may be a by-product of normal aging, or may occur with several other conditions. In this picture, we see how amyloidosis can affect the skin as nodular deposits on the fingers.

References

Gertz MA. Amyloidosis. In: Goldman L, Schafer AI, eds. Goldman-Cecil Medicine. 26th ed. Philadelphia, PA: Elsevier; 2020:chap 179.

Lachmann HJ, Sharpley FA. Amyloidosis. In: Firestein GS, Budd RC, Gabriel SE, Koretzky GA, McInnes IB, O'Dell JR, eds. Firestein & Kelley's Textbook of Rheumatology. 11th ed. Philadelphia, PA: Elsevier; 2021:chap 123.

Last reviewed November 5, 2021 by 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, Brenda Conaway, Editorial Director, and the A.D.A.M. Editorial team..

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