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level: Amyloidosis

Questions and Answers List

level questions: Amyloidosis

QuestionAnswer
Misfolded protein that deposits in extracellular spaceAmyloid
Beta pleated sheets, Congo red staining and apple green birefringence under polarized lightFeatures of amyloid proteins
Congo red stainStaining used to visualize amyloid
Apple green birefringenceCharacteristic finding under polarized light
Beta pleated sheetProtein configuration of amyloid
Overproduction of immunoglobulin light chain; associated with plasma cell dyscrasia (eg. multiple myeloma)Primary amyloidosis
Primary amyloidosisAL amyloid
Secondary amyloidosisAA amyloid
Acute phase reactant elevated in chronic inflammatory states, malignancy and Familial Mediterranean FeverSerum amyloid-associated (SAA) protein
Autosomal recessive neutrophil dysfunction; Neutrophils abnormally activated -> inflammation of serosal surfaces; Presents as fever and serositisFamilial Mediterranean Fever (FMF)
Systemic deposition of AA amyloid derived from serum amyloid-associated proteinSecondary amyloid
KidneysMost common organ involved in systemic amyloidosis
Tissue biopsyDiagnosis of amyloidosis
Abdominal fat pad, RectumCommon sites for tissue biopsy in amyloidosis
Nephrotic syndrome, restrictive cardiomyopathy, arrhythmia, tongue enlargement, malabsorption, hepatosplenomegalyClinical findings of systemic amyloidosis
Senile cardiac amyloidosisNon-mutated serum transthyretin
Familial amyloid cardiomyopathyMutated serum transthyretin
Type 2 Diabetes MellitusAmylin deposition in pancreatic islets
Alzheimers disease; derived from beta amyloid precursor proteinAmyloid beta
Location of gene for beta APPChromosome 21
Associated with early onset Alzheimer's Disease (age 40)Down Syndrome
Dialysis-associated amyloidosis; accumulates in jointsB2 microglobulin
Medullary carcinoma of the thyroidCalcitonin