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Amyloid light-chain (AL) amyloidosis, also known as primary amyloidosis, is the most common form of systemic amyloidosis. [1] The disease is caused when a person's antibody -producing cells do not function properly and produce abnormal protein fibers made of components of antibodies called light chains .
Amyloidosis is a group of diseases in which abnormal proteins, known as amyloid fibrils, build up in tissue. [4] There are several non-specific and vague signs and ...
AA amyloidosis is a form of amyloidosis, a disease characterized by the abnormal deposition of fibers of insoluble protein in the extracellular space of various tissues and organs. In AA amyloidosis, the deposited protein is serum amyloid A protein (SAA), an acute-phase protein which is normally soluble and whose plasma concentration is highest ...
The initial presentation of PrP systemic amyloidosis is chronic diarrhea in the fourth decade of life, which may be misdiagnosed as Crohn's disease or irritable bowel syndrome. [1] Sensory and/or autonomic neuropathy follows. In one case, the disease caused optic nerve atrophy and visual disturbances in a Japanese woman. [2]
Amyloid shows up as homogeneous pink material in lamina propria and around blood vessels. 20× magnification. Amyloids are aggregates of proteins characterised by a fibrillar morphology of typically 7–13 nm in diameter, a β-sheet secondary structure (known as cross-β) and ability to be stained by particular dyes, such as Congo red. [1]
Aducanumab, sold under the brand name Aduhelm, is a monoclonal antibody designed to treat Alzheimer's disease. It is a monoclonal antibody that targets aggregated forms (plaque) of amyloid beta (Aβ) found in the brains of people with Alzheimer's disease to reduce its buildup. [10] It was developed by Biogen and Eisai. [11] Aducanumab is given via intravenous infusion. [5] Aducanumab was ...
Macular amyloidosis is a cutaneous condition characterized by itchy, brown, rippled macules usually located on the interscapular region of the back. [ 4 ] : 521 Combined cases of lichen and macular amyloidosis are termed biphasic amyloidosis, and provide support to the theory that these two variants of amyloidosis exist on the same disease ...
The shoulder pad sign is an enlargement of the anterior shoulder due to amyloid deposition in periarticular soft tissue. [1] [2] [3] This type of infiltration is not common, but it is pathognomonic for AL amyloidosis. [1] [2] [3] Analysis of the amyloid protein has demonstrated that it has a kappa III Ig light chain structure. [1]
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