Radiograph showing carpal tunnel syndrome in a patient with dialysis-associated amyloidosis. Long-term hemodialysis often results in carpal tunnel syndrome with pain involving the shoulders, hands, wrists, hips, and knees. Cystic radiolucen-cies are common in the carpal bones. Pathologic fractures have occurred from large amyloid deposits. The major component of the amyloid is ^-microglobulin. (From Gertz and Kyle ; with permission.)
of bladder and gastrointestinal function are common. Late onset may occur with the development of symptoms in the seventh or eighth decade of life. The nephropathic form is most often caused by familial Mediterranean fever. This form affects persons of Mediterranean descent and is characterized by recurrent episodes of fever and abdominal pain that begin in childhood.
Familial amyloidosis involving the kidneys has been reported by Ostertag  and others [20-22]. Families with apolipoprotein A1 mutation, as well as mutations in the fibrinogen a-chain gene, have been recognized. On presentation, patients with renal involvement exhibit hypertension and mild renal insufficiency that progresses to endstage renal failure. The amyloid deposits have mutations in the fibrino-gen a-chain gene. This form of amyloidosis is autosomal dominant. No peripheral neuropathy develops, and the onset of renal disease occurs in the fifth to seventh decades of life. The mutation consists of the substitution of glutamic acid for valine at position 526 of the fibrinogen chain. A mutation in fibrinogen has been described at position 554 [23,24]. A rare form of inherited secondary amyloidosis produces nephropathy, deafness, and urticaria. This form has been referred to as the Muckle-Wells syndrome . (Adapted from Kyle and Gertz .)
RATE OF AMYLOIDOSIS ((¡»-MICROGLOBULIN) WITH DIALYSIS
Years of dialysis
Patients with amyloidosis, %
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