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Rapid Generation of Amyloid from Native Proteins In vitro
Published on: December 5, 2013
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AL Amyloidosis Presenting With Crescentic Glomerulonephritis.
Ann A Wang1, Yashpal S Kanwar2, Vikram Aggarwal3
1Graduate Medical Education, Northwestern University Feinberg School of Medicine, Chicago, IL.
Kidney Medicine
|August 17, 2021
Summary
Kidney amyloidosis can mimic rapidly progressive glomerulonephritis. This case highlights light chain (AL) amyloidosis presenting with crescent formation, emphasizing the need for thorough investigation in nephrotic syndrome.
Area of Science:
- Nephrology
- Pathology
- Immunology
Background:
- Kidney amyloidosis commonly causes nephrotic-range proteinuria.
- Crescentic glomerulonephritis is a rare presentation of kidney amyloidosis, usually linked to AA amyloidosis.
Observation:
- A previously healthy man in his 70s presented with severe acute kidney injury, nephrotic-range proteinuria, and nephritic urinary sediment.
- Initial tests for rapidly progressive glomerulonephritis causes were negative.
- Kidney biopsy revealed active crescents and Congo red-positive staining, with amyloid fibrils confirmed via electron microscopy.
Findings:
- Urinary protein electrophoresis identified monoclonal lambda light chains.
- Bone marrow biopsy confirmed the diagnosis of kidney AL amyloidosis.
Implications:
- This case demonstrates that AL amyloidosis can manifest with clinical and histological features suggestive of rapidly progressive glomerulonephritis.
- Highlights the importance of considering AL amyloidosis in patients with unexplained crescentic glomerulonephritis and nephrotic syndrome.
Keywords:
AL amyloidosisglomerular diseasekidney amyloidosiskidney biopsyrapidly progressive glomerulonephritisMore Related Videos
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