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level: Amyloidosis

Questions and Answers List

level questions: Amyloidosis

Question	Answer
Misfolded protein that deposits in extracellular space	Amyloid
Beta pleated sheets, Congo red staining and apple green birefringence under polarized light	Features of amyloid proteins
Congo red stain	Staining used to visualize amyloid
Apple green birefringence	Characteristic finding under polarized light
Beta pleated sheet	Protein configuration of amyloid
Overproduction of immunoglobulin light chain; associated with plasma cell dyscrasia (eg. multiple myeloma)	Primary amyloidosis
Primary amyloidosis	AL amyloid
Secondary amyloidosis	AA amyloid
Acute phase reactant elevated in chronic inflammatory states, malignancy and Familial Mediterranean Fever	Serum amyloid-associated (SAA) protein
Autosomal recessive neutrophil dysfunction; Neutrophils abnormally activated -> inflammation of serosal surfaces; Presents as fever and serositis	Familial Mediterranean Fever (FMF)
Systemic deposition of AA amyloid derived from serum amyloid-associated protein	Secondary amyloid
Kidneys	Most common organ involved in systemic amyloidosis
Tissue biopsy	Diagnosis of amyloidosis
Abdominal fat pad, Rectum	Common sites for tissue biopsy in amyloidosis
Nephrotic syndrome, restrictive cardiomyopathy, arrhythmia, tongue enlargement, malabsorption, hepatosplenomegaly	Clinical findings of systemic amyloidosis
Senile cardiac amyloidosis	Non-mutated serum transthyretin
Familial amyloid cardiomyopathy	Mutated serum transthyretin
Type 2 Diabetes Mellitus	Amylin deposition in pancreatic islets
Alzheimers disease; derived from beta amyloid precursor protein	Amyloid beta
Location of gene for beta APP	Chromosome 21
Associated with early onset Alzheimer's Disease (age 40)	Down Syndrome
Dialysis-associated amyloidosis; accumulates in joints	B2 microglobulin
Medullary carcinoma of the thyroid	Calcitonin