primary amyloidosis

amyloidosis /am·y·loi·do·sis/ (am″ĭ-loi-do´sis) a group of conditions characterized by the accumulation of insoluble fibrillar proteins (amyloid) in various organs and tissues such that vital function is compromised. The associated disease states may be inflammatory, hereditary, or neoplastic, and deposition can be local, generalized, or systemic. The most widely used classification is based on the chemistry of the amyloid fibrils and includes primary (AL), secondary (AA), and familial forms.

AA amyloidosis secondary a.

AL amyloidosis primary a.

ATTR amyloidosis the most common form of familial amyloidosis (q.v.), associated with mutations of the gene encoding transthyretin.

familial amyloidosis any inherited form of amyloidosis; usually used to denote any of various systemic autosomal dominant disorders of amyloid deposition that involve the nervous system. The most common form, ATTR amyloidosis, is associated with mutations of the transthyretin protein, but rare mutations of other proteins can also be a cause. The term can also, less commonly, be said to include several types of secondary amyloidosis in which the deposition is associated with an inherited disease (e.g., familial Mediterranean fever). Subclassifications are based on clinical presentation and biochemical composition of the fibrils deposited; originally they were distinguished on the basis of kinship. See also familial amyloid polyneuropathy, under polyneuropathy.

hemodialysis-associated amyloidosis that occurring in patients on long-term hemodialysis, caused by the deposition of beta2-microglobulin, which cannot be removed from the blood by hemodialysis, in the joints, synovial membranes, and tendon sheaths. Manifestations include carpal tunnel syndrome and arthritis.

hereditary amyloidosis familial a.

primary amyloidosis a systemic form in which the deposited fibrillar material is AL amyloid; it may be due to either aberrant synthesis or processing of immunoglobulin light chains. It is associated with tumors or dyscrasias of immunoglobulin-producing plasma cells and involves some combination of the skin and subcutaneous tissue, nerves, liver, spleen, heart, kidney, intestine, and tongue. Called also AL a..

reactive systemic amyloidosis secondary a.

renal amyloidosis amyloid deposits in the kidneys; in the primary type the fibrils are mainly AL amyloid, and in secondary types they are AA amyloid. Secondary types may accompany inflammatory disorders, chronic infectious diseases, or neoplastic diseases.

secondary amyloidosis that in which AA amyloid is deposited, and which occurs secondary to a chronic inflammatory condition, either infectious or noninfectious. It usually involves the liver, spleen, and kidneys.

primary amyloidosis

A rare form of amyloidosis that is sometimes hereditary and is characterized by amyloid deposits in the heart, tongue, gastrointestinal tract, and skeletal muscle.

primary amyloidosis.

See amyloidosis.

amyloidosis (am´iloidō´sis),

n a condition in which amyloid, a glycoprotein, is deposited intercellularly in tissues and organs. Four types of amyloidosis are recognized, two of which, primary amyloidosis and amyloid tumor, frequently produce nodules in the tongue and gingiva.

Amyloidosis.

amyloidosis, primary,

n a type occurring without a known predisposing cause. Amyloid deposits are found in the tongue, lips, skeletal muscles, and other mesodermal structures. The disease may be manifested by polyneuropathy, purpura, hepatosplenomegaly, heart failure, and the nephrotic syndrome.

amyloidosis, secondary,

n a type occurring secondary to chronic diseases such as tuberculosis, leprosy, rheumatoid arthritis, multiple myeloma, and prolonged bacterial infections. Amyloid deposits are found in parenchymal organs. The disease is usually manifested by proteinuria and hepatosplenomegaly.

Patient discussion about primary amyloidosis.

Q. How fast can primary amyloidosis spread? And what is the average survival rate for this disease?

A. Your best bet for one-stop shopping for amyloidosis information on the net is http://www.amyloidosis.org. There is also a Yahoo group named amyloidosis and also a mailing list named amyloid hosted by the Association of Cancer Online Resources. The amyloid list has around 500 subscribers, amyloidosis Yahoo group has maybe half that many. Face-to-face support for patients and caregivers is also available in most large metropolitan areas two or three times a year. See http://www.amyloidosissupportgroups.com for details.

To join the amyloid list, see http://www.acor.org/amyloid.html

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amyloid amyloidosis atrial systolic failure ATTR amyloidosis familial amyloidosis hepatic amyloidosis hereditary amyloidosis immunosecretory disorders Raccoon Eye secondary amyloidosis	population and has long been thought to be a precursor of serious diseases, such as multiple myeloma, primary amyloidosis, and Waldenstrom macroglobulinemia. MGUS not linked to as many diseases by Medical Laboratory Observer Therefore, we declared a diagnosis of primary amyloidosis of the right external auditory canal and middle ear. Amyloidosis of the external auditory canal and middle ear: unusual ... by O'Sullivan, Timothy J. / Ear, Nose and Throat Journal Abstract: Cardiac involvement is the most important prognostic factor in primary amyloidosis (AL). Hypertrophic cardiomyopathy and symptomatic conduction system disease ... by Woodlock, Timothy J. / Southern Medical Journal	prim- Prima prima facie prima facie rights primal primal scene Primal Scream Therapy Primal Therapy primaquine primaquine phosphate primary primary abscess primary acquired melanosis primary action primary adhesion primary adrenocortical insufficiency Primary Aerosolisation primary afferent fiber primary alcohol primary aldosteronism primary alveolar hypoventilation primary amebic meningoencephalitis primary amenorrhea primary amputation primary amyloid primary amyloidosis primary anesthesia primary anesthetic primary apnea primary atelectasis primary atypical pneumonia primary autonomic dysfunction primary bilateral micronodular hyperplasia primary biliary cirrhosis primary brain tumor primary brain vesicle primary bronchial buds primary bronchus primary carcinoma primary care primary care case management Primary Care Certificate in Homeotherapeutics primary care exception primary care network primary care physician primary care trust primary caries primary cataract primary cause of death primary cell primary cell culture	primary alcohol primary alcohol primary alcohol Primary alcohols Primary alcohols Primary alcohols Primary alcohols primary aldosteronism primary aldosteronism primary aldosteronism Primary Alerting System Primary Alternate Contingency Emergency Primary alveolar hypoventilation Primary alveolar hypoventilation primary amebic meningoencephalitis primary amebic meningoencephalitis primary amebic meningoencephalitis primary amenorrhea primary amenorrhea primary amenorrhea Primary amine Primary amine Primary amine Primary amines Primary amines Primary amines Primary amoebic meningoencephalitis Primary amputation Primary amputation primary amyloid primary amyloidosis Primary and Ambulatory Care Division Primary and Community Care Audit Group Primary and Mass Education Division Primary and Rural Health Care Systems Branch Primary and Secondary Primary and secondary explosives Primary and Secondary Qualities Primary Androgen Deprivation Therapy primary anesthesia primary anesthetic Primary Angiitis of the Central Nervous System Primary Angioplasty in Myocardial Infarction Trial Primary Angle Closure Primary Angle Closure Glaucoma Primary anorgasmia Primary anorgasmia Primary Antenna Interface Controller primary apnea primary apnea primary apnea Primary Application Window Primary Appraisal of Smoking Cessation Inventory primary arc primary area primary area primary area primary area primary area Primary Area of Technical Competence Primary Area Switch Locator

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amyloid
amyloidosis
atrial systolic failure
ATTR amyloidosis
familial amyloidosis
hepatic amyloidosis
hereditary amyloidosis
immunosecretory disorders
Raccoon Eye
secondary amyloidosis

population and has long been thought to be a precursor of serious diseases, such as multiple myeloma, primary amyloidosis, and Waldenstrom macroglobulinemia.

MGUS not linked to as many diseases by Medical Laboratory Observer

Therefore, we declared a diagnosis of primary amyloidosis of the right external auditory canal and middle ear.

Amyloidosis of the external auditory canal and middle ear: unusual ... by O'Sullivan, Timothy J. / Ear, Nose and Throat Journal

Abstract: Cardiac involvement is the most important prognostic factor in primary amyloidosis (AL).

Hypertrophic cardiomyopathy and symptomatic conduction system disease ... by Woodlock, Timothy J. / Southern Medical Journal

Primal Scream Therapy