Zusammenfassung der Ressource
Amyloidosis
- definition
- deposition of amyloid in the Interstitial tissues +wall of BVs
- Structure
- Chemical
- AA
- AL
- Transthyretin
- B2 microglubulin
- b amyloid protein
- physical
- structure
- 95%=non branching fibrills 5%=p components +glycoprotein
- non branching fibrills in the shape of crossed B pleated sheets
- plasma protein primary amyloidosis light chain Igs
- no Igs liver 2ry amyloidosis
- normal serum protein transports thyroxin and retinol senile systemic amyloidosis +familial amyloid polyneropathy
- long term dialysis synovium+joints+tendon sheath.
- alzheimer
- Classification
- primary
- secondary
- Myeloma pts serum has Bence Jones prts(kappa ,lambda)
- all the other diseases
- pathogenesis: defective degradation in bence jones prts
- pathogenesis: liver produces SAA under the effect of IL1 which leads to insoluble SAA
- site
- localized in a single organ
- systematic in multiple organs
- clinical feature in different organs :
- generally : weakness ,weight loss, fatigue
- heart: cardiomyopathy+arrhythmia
- in kidney: renal failure
- in liver:hepatosplenomegaly
- diagnosis: must be done by biopsy stained by Congo red/in generalizes most cases have rectal and gingival amyloid.
- sites: heart,skin,peripheral nerves,tongue,respiratory
- sites:kidney,liver,spleen, LNs,adrenal,thyroid.