Systemic immunoglobulin light chain amyloidosis
نویسندگان
چکیده
منابع مشابه
Hereditary systemic immunoglobulin light-chain amyloidosis.
Several members of a family died from renal failure as a result of systemic amyloidosis. Extensive studies to detect previously documented gene mutations associated with amyloidosis failed to identify a causative factor. In search of the genetic basis for this syndrome, amyloid fibrils were isolated from renal tissue of a member of the kin who died while on renal dialysis. Amino acid sequencing...
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Background: Maintenance of a good nutritional status is associated with prolonged survival in many chronic diseases. To date, the nutritional status of outpatients with immunoglobulin light-chain (AL) amyloidosis has not been evaluated. Objective: The aims of this study were to obtain information regarding the nutritional status of AL amyloidosis outpatients and to investigate its prognostic ro...
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BACKGROUND Primary immunoglobulin light chain amyloidosis (AL amyloidosis) is a plasma cell disorder which mainly affects heart, kidneys, liver, and peripheral nervous system. Cases of atypical AL amyloidosis presented as spontaneous vertebral compression fractures have been rarely reported, and data about the management and clinical outcomes of the patients are scarce. METHODS Herein, we pre...
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The goal of this study was to investigate the frequency of use of light-chain variable region (IGVL) genes among patients with systemic (ALS) and localized (ALL) amyloidosis and to assess for associations between IGVL gene usage and organ tropism. We evaluated clinic charts from 821 AL patients seen at the Mayo Clinic who had bone marrow, fat pad, and solid organ tissue samples typed by liquid ...
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Secondary amyloidosis can complicate chronic inflammatory autoimmune diseases. However, the clinical findings of primary amyloidosis may mimic those of primary rheumatologic disorders. We present the case of a 53-year-old woman who presented with dystrophic nail changes, dry eyes, bilateral carpal tunnel syndrome, Raynaud's phenomenon, and high titer positive nucleolar pattern antinuclear antib...
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ژورنال
عنوان ژورنال: Nature Reviews Disease Primers
سال: 2018
ISSN: 2056-676X
DOI: 10.1038/s41572-018-0034-3