FAMILIAL AMYLOIDOSIS OF OSTERTAG

  • 1 January 1982
    • journal article
    • research article
    • Vol. 51  (201) , 25-32
Abstract
A 23 yr old Englishman presented with keratoconjunctivitis sicca and had systemic amyloidosis. Five members of his family in 2 generations also had nonneuropathic amyloid particularly affecting the kidneys. This conforms to the Ostertag type of hereditary amyloidosis. Amyloid deposits in the proband showed permanganate-sensitive Congophilia and positive immunofluorescence staining for P component, but were negative for amyloid A and prealbumin. The fibril protein in this patient was apparently immunochemically distinct from the amyloid fibrils previously characterized.