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AMYLOIDOSIS.

aMYLOIDOSIS

QuestionAnswer
Whats is amyloidosis? misfolded protein tli.it deposits in the extracellular space(OUTSIDE), thereby damaging tissues.
Kind of Protiens deposited and how does it look like? B-pleated sheet configuration Congo red staining and apple-green birefringence when viewed microscopically under polarized light. Deposition around blood vessels
2 kinds of amyloidosis? Systemic and localized Systemic is primary and secondary
What is primary systemic amyloidosis? In plasma cell dyscrasias (e.g.,abnormalities of the plasma cells multiple myeloma)there is an access of light chain production of the immunoglobulins. There get misfolded into AL amyloid and deposited in the system
What is secondary systemic amyloidosis? Chronic inflamation (or malignancy and FAMILIAM MEDITERANEAN FEVER)releases acute phase reactants like SAA -->serum amyloid-associated protein--> later is deposited as AA amyloid systemically
Describe what familial mediterranean fever is. FMF is due to a dysfunction of neutrophils (autosomal recessive) in Mediterranean ppl. Presents with fever and acute serosal inflammation WITHOUT INFECTION Serosa inflamation of heart-MI Serosa inflamation of abdomen-apendicitis High SAA-->AA deposi
Clinical findings of systemic amyloidosis 1. Nephrotic syndrome;KIDNEY is the most common organ involved. and causes large loss of protien 2. Restrictive cardiomyopathy or arrhythmia (improper pumping)) 3. Tongue enlargement, malabsorption, and hepatosplenomegalv
Diagnosis and treatment of amyloidosis? Diagnosis requires tissue biopsy. Abdominal fat pad and rectum are easily accessible biopsy targets. E. Damaged organs must be transplanted. Amyloid cannot be removed.
What is localized amyloidosis? localized to a single organ.
Amyloidosis in Senile cardiac amyloidosis 1. Non-mutated scrum transthyretin deposits in the heart. This is one of the most common protien in the blood and gets deposited in the heart Usually asymptomatic; present in 25% of individuals > 80 years of age
Amyloidosis in Familial amyloid cardiomyopathy 1. Mutated serum transthyretin(one of the most common blood protien) deposits in the heart leading to restrictive ca rd iomyopathy. 2. 5% of African Americans carry the mutated gene.
Amyloidosis in Diabetes11 Noiv-insu 1 in-dependent diabetes mellitus (type II) i, Aniylin (derived from insulin) deposits in the islets of the pancreas,
Amyloidosis in Alzhemiers, dialysis and thyroid 1. A|i amyloid (derived from (J-amyloid precursor protein) deposits in the brain forming amyloid plaques, 2.Dialysis-associated -microglobulin deposits in joints, 3. Medullary carcinoma of the thyroid- Calcitonin deposits within the tumor
Created by: atayal
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