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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 .
AL amyloidosis occurs in about 3–13 per million people per year and AA amyloidosis in about two per million people per year. [2] The usual age of onset of these two types is 55 to 60 years old. [2] Without treatment, life expectancy is between six months and four years. [2]
Wild-type transthyretin amyloid (WTTA), also known as senile systemic amyloidosis (SSA), [1] is a disease that typically affects the heart and tendons of elderly people. It is caused by the accumulation of a wild-type (that is to say a normal ) protein called transthyretin .
Familial amyloid polyneuropathy, also called transthyretin-related hereditary amyloidosis, transthyretin amyloidosis abbreviated also as ATTR (hereditary form), or Corino de Andrade's disease, [1] is an autosomal dominant [2] neurodegenerative disease.
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]
Granulomatosis with polyangiitis 50-60% have ophthalmologic manifestations, which can be a presenting feature in a minority of patients. Orbital disease is the most common manifestation, and may result in proptosis, restrictive ophthalmopathy, chronic orbital pain, and in chronic cases, orbital retraction syndrome and intractable socket pain.
The disorder is primarily associated with eye, skin, and cranial nerve symptoms with the onset of symptoms appearing between the thirties and fifties. [3] The most common characteristic is type II lattice corneal dystrophy with other signs such as polyneuropathy, dermatochalasis, open-angle glaucoma, bilateral progressive facial paralysis, cutis laxa, skin fragility with ecchymosis, facial ...
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