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🌱 來自: amyloidosis

overview of amyloidosis

• Types of Amyloidosis: Extracellular tissue deposition of fibrils made up of low molecular weight subunits from various proteins.
• Subunit Proteins: Derived from soluble precursors undergoing conformational changes leading to beta-pleated sheet configuration.
• 38 Protein Precursors: Known for forming amyloid fibrils.
• Major Forms:
  • AL Amyloidosis: Result of protein deposition from immunoglobulin light chain fragments.
  • Transthyretin Amyloidosis (ATTR): Caused by hereditary mutations or aging-related factors.
  • AA Amyloidosis: Common in resource-limited regions, linked to chronic inflammation.
  • Additional Forms: Dialysis-related, hereditary, age-related, organ-specific amyloidosis, etc.
• Clinical Manifestations: Vary based on amyloid type and distribution.
• Diagnosis: Tissue biopsy essential for confirmation. Different biopsy sites based on organ involvement.
• Monoclonal Protein Testing: Biopsy-proven amyloidosis with plasma cell disorder may not need further testing. Monoclonal protein presence tested via electrophoresis and immunofixation.
• Treatment: Varies depending on fibril production cause, targeting underlying disorders.
• AL Amyloidosis: Targets plasma cell dyscrasia.
• AA Amyloidosis: Focuses on underlying infections or inflammatory issues.
• Dialysis-Related Amyloidosis: Adjusts dialysis mode or considers transplantation.
• Hereditary Amyloidoses: Liver transplantation may be effective.
• Therapies: Aim to decrease TTR production and clear amyloid deposits.