Sheeza Basharatalmost 3 years ago

AMYLOIDOSIS

It is a heterogeneous disease that results from the deposition of toxic insoluble beta-sheet fibrillar protein aggregates in different tissues. ⠀ ⠀ ✅It can be acquired or hereditary.⠀ ⠀ ⏩It is classified according to systemic, hereditary, central nervous system, ocular, and localized etiology.⠀ ⠀ ⏩The most common causes are immunoglobulin-light-chain relate amyloidosis (AL), ATTR amyloidosis, and reactive amyloidosis (AA) due to chronic inflammatory diseases like chronic infections and rheumatoid arthritis. ⠀ ⠀ ⏩Polypeptides can adopt alternative misfolded states, making them prone to aggregation. ⠀ ⠀ ✅Low pH, increased temperatures, limited proteolysis, osmolytes, and metal ions can alter the tridimensional structure of proteins shifting the equilibrium towards the amyloidogenic state. ⠀ ⠀ ⏩The mechanism of tissue damage in amyloidosis involves alteration of tissue architecture, interaction with cell surface receptors, inflammation elicited by the amyloid protein deposition, oxidative stress, and apoptosis activation.⠀ ⠀ ✅Diagnostic and differentiating feature is apple-green birefringence of amyloid on congo red staining. ⠀ ⠀ ⏩Amyloid has an amorphous eosinophilic appearance, when viewed with hematoxylin and eosin staining. ⠀ ⠀ ⏩Systemic amyloidosis (AA) can lead to heart failure with left ventricular hypertrophy on echocardiogram with standard or low voltage electrocardiogram. ⠀ ⠀ ⏩Hepatomegaly, nephrotic syndrome, macroglossia, orthostatic hypotension, ecchymosis, and autonomic and peripheral neuropathy can be present. ⠀ ⠀ ⏩Other physical exam findings include hypertrophied shoulder pads from amyloid deposition, amyloid purpura, and raccoon eyes secondary to factor-X deficiency and prolonged PT, PTT.⠀ ⠀ ⏩Diagnosis⠀ ✅The subcutaneous abdominal fat aspirate⠀ ✅Biopsy of minor salivary glands⠀