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Familial Amyloidosis of Ostertag

J. G. LANHAM, MARGARET L. MELTZER, F. C. DE BEER, G. R. V. HUGHES, M. B. PEPYS
DOI: http://dx.doi.org/ 25-32 First published online: 1 January 1982

Abstract

SUMMARY A 23 year old Englishman presented with keratoconjunctivitis sicca and was found to have systemic amyloidosis. Five members of his family in two generations also had non-neuropathic 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. These observations suggested that the fibril protein in this patient was immunochemically distinct from the amyloid fibrils characterized hitherto.