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Related Concept Videos

Amyloid Fibrils03:03

Amyloid Fibrils

Amyloid fibrils are aggregates of misfolded proteins.  Under most circumstances, misfolded proteins are either refolded by chaperone proteins or degraded by the proteasome. However, in the case of a mutation or a disease, these proteins can accumulate to form large clusters and often further assemble to form elongated fibers, called fibrils. 
Amyloid deposits were observed as early as 1639 in the liver and the spleen.   In 1854, Rudolph Virchow performed iodine staining, normally used to...
Amyloid Fibrils03:03

Amyloid Fibrils

Amyloid fibrils are aggregates of misfolded proteins.  Under most circumstances, misfolded proteins are either refolded by chaperone proteins or degraded by the proteasome. However, in the case of a mutation or a disease, these proteins can accumulate to form large clusters and often further assemble to form elongated fibers, called fibrils. 
Amyloid deposits were observed as early as 1639 in the liver and the spleen.   In 1854, Rudolph Virchow performed iodine staining, normally used to...
Radiological Investigation III: Pulmonary Angiogram and PET Scan01:13

Radiological Investigation III: Pulmonary Angiogram and PET Scan

Radiological investigations are paramount in the diagnosis and management of various pulmonary diseases. Two essential investigations are the Pulmonary Angiogram and the Positron Emission Tomography (PET) Scan.
Pulmonary Angiogram
A Pulmonary Angiogram is an invasive procedure involving injecting a contrast medium through a catheter threaded into the pulmonary artery or the right side of the heart to visualize the pulmonary vasculature. Computed Tomography (CT) scans have mainly replaced this...
Imaging Studies VII: Vascular Imaging01:19

Imaging Studies VII: Vascular Imaging

DefinitionRenal angiography, also known as renal arteriography, is an imaging technique used to obtain a comprehensive view of blood flow and the vascular structure of blood vessels in the kidneys and surrounding areas.PurposeRenal angiography detects blood vessel abnormalities in the kidneys, such as aneurysms, stenosis, thrombosis, vascular tumors, and renal artery stenosis. It evaluates kidney function and guides interventional treatments like angioplasty or stent placement.Pre-Procedure...

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Related Experiment Video

Updated: Jul 12, 2026

Imaging Amyloid Tissues Stained with Luminescent Conjugated Oligothiophenes by Hyperspectral Confocal Microscopy and Fluorescence Lifetime Imaging
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Multimodality imaging features of systemic amyloidosis: a case report.

Yi Yu1,2, Zhi-Chao Li3, Guang-Yin Li4

  • 1Department of Ultrasound, Shanghai Chest Hospital, Shanghai Jiao Tong University School of Medicine, 241 Huaihai Road, Shanghai, 200030, China. yuyichesthospital@163.com.

BMC Cardiovascular Disorders
|January 3, 2025
PubMed
Summary

Systemic light chain amyloidosis (AL) is a rare disease often missed in early stages. Multimodal imaging, including echocardiography and biopsy, is crucial for accurate diagnosis and timely treatment of AL amyloidosis.

Keywords:
Case reportLight chainMultimodality imagingMyocardial amyloidosisSystemic

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Area of Science:

  • Cardiology
  • Hematology
  • Medical Imaging

Background:

  • Systemic light chain amyloidosis (AL) is a rare, aggressive plasma cell disorder with significant morbidity and mortality.
  • Gastrointestinal and myocardial involvement are common but can lead to delayed diagnosis due to non-specific symptoms and challenges with routine imaging.
  • Early and accurate diagnosis is critical for initiating effective treatment and improving patient outcomes.

Observation:

  • A 68-year-old male presented with prolonged diarrhea and dysphagia, but no dyspnea.
  • Echocardiography showed "ground glass" myocardial hypertrophy with reduced global longitudinal strain (-9.00%), despite normal ejection fraction (51%).
  • Cardiac MRI was negative for gadolinium enhancement, complicating diagnosis.

Findings:

  • Elevated serum free light chains (κ and λ) and abnormal urinary protein quantification confirmed AL amyloidosis.
  • Abdominal fat biopsy using electronic and immunoelectron microscopy provided definitive histopathological diagnosis.
  • Multimodal imaging, including echocardiography and biopsy, revealed widespread organ involvement (heart, kidneys, GI tract, nervous system), classifying the patient as Stage III (Mayo 2012).

Implications:

  • This case highlights the diagnostic challenges of AL amyloidosis, particularly when cardiac MRI is negative for enhancement.
  • Reduced global longitudinal strain on echocardiography and abnormal ECG findings are vital indicators for early myocardial amyloidosis detection.
  • Comprehensive utilization of multimodal imaging and histopathology is essential for accurate diagnosis and management of systemic AL amyloidosis.