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Familial amyloidosis of Ostertag.

Lanham, JG; Meltzer, ML; De Beer, FC; Hughes, GR; Pepys, MB; (1982) Familial amyloidosis of Ostertag. Q J Med , 51 (201) pp. 25-32.

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Abstract

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.

Type: Article
Title: Familial amyloidosis of Ostertag.
Location: England
Keywords: Adult, Amyloid, Amyloidosis, Humans, Keratoconjunctivitis, Kidney Diseases, Kidney Glomerulus, Male, Pedigree, Rectum, Salivary Glands, Minor, Serum Amyloid P-Component
UCL classification: UCL > Provost and Vice Provost Offices
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Medical Sciences
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Medical Sciences > Div of Medicine
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Medical Sciences > Div of Medicine > Inflammation
URI: http://discovery.ucl.ac.uk/id/eprint/1552136
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