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Related Concept Videos

Amyloid Fibrils03:03

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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. 
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Methods for Detecting Cytotoxic Amyloids Following Infection of Pulmonary Endothelial Cells by Pseudomonas aeruginosa
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Amyloid in the Lung.

Misbah Baqir1, Anja C Roden2, Teng Moua1

  • 1Division of Pulmonary and Critical Care Medicine, Mayo Clinic, Rochester, Minnesota.

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|April 13, 2020
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Summary
This summary is machine-generated.

Pulmonary amyloidosis involves abnormal protein deposits in the lungs, often linked to AL and AA amyloid subtypes. Prognosis depends on overall organ involvement, with multisystemic disease posing the highest risk.

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Area of Science:

  • Pulmonology
  • Pathology
  • Radiology

Background:

  • Amyloidosis is characterized by misfolded protein deposition, leading to organ dysfunction.
  • Pulmonary manifestations are nonspecific and associated with amyloid protein subtypes like AL and AA.
  • Clinical presentation often reflects other organ involvement more than lung-specific symptoms.

Purpose of the Study:

  • To summarize the pulmonary manifestations of amyloidosis.
  • To describe associated radiologic findings and clinical implications.
  • To discuss the prognostic factors in pulmonary amyloidosis.

Main Methods:

  • Review of literature on pulmonary amyloidosis.
  • Analysis of radiologic findings in affected patients.
  • Correlation of clinical signs with organ involvement.

Main Results:

  • Radiologic findings include infiltrates, nodules, cysts, and tracheobronchial abnormalities.
  • Lymphadenopathy, calcification, and pleural effusions may also be present.
  • Therapy is directed by clinical signs, often secondary to systemic diseases.

Conclusions:

  • Pulmonary involvement in amyloidosis is variable and nonspecific.
  • Prognosis is largely determined by the extent of systemic organ involvement.
  • Multisystemic disease carries the highest morbidity and poorest long-term outlook.