Transthyretin-associated neuropathic amyloidosis. Pathogenesis and treatment.

  title={Transthyretin-associated neuropathic amyloidosis. Pathogenesis and treatment.},
  author={Ernst Hund and Reinhold Paul Linke and Friedrich Willig and Armin Grau},
  volume={56 4},
Hereditary amyloidoses form a clinically and genetically heterogeneous group of autosomal dominantly inherited diseases characterized by the deposit of insoluble protein fibrils in the extracellular matrix. They typically present with polyneuropathy, carpal tunnel syndrome, autonomic insufficiency, and cardiomyopathy and gastrointestinal features, occasionally accompanied by vitreous opacities and renal insufficiency. Other phenotypes are characterized by nephropathy, gastric ulcers, cranial… CONTINUE READING
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