CLINICAL FEATURES
In
systemic forms of amyloidosis, the
kidney is the most commonly involved organ, and patients may experience
nephrotic syndrome and/or progressive renal failure. Cardiac involvement may
cause restrictive cardiomyopathy and conduction dis-turbances. Other clinical
features include hepatosplenomegaly and involvement of the gastrointestinal
tract, which may produce tongue enlargement (macroglossia, primarily in AL
type) and malabsorption.
Diagnosis in
systemic forms of amyloidosis can be established with biopsy of therectal
mucosa, gingiva, or the abdominal fat pad; Congo red stain shows apple green
birefringence under polarized light of amyloid deposits. The prognosis of
systemic amyloidosis is poor. AL amyloidosis is diagnosed by serum and urinary
protein electrophoresis and immunoelectrophoresis. Proteomic analysis is
another diag-nostic tool.
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