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Amyloidosis in Familial Mediterranean Fever:  An Independent Genetically Determined Character

HARRY HELLER, M.D.; EZRA SOHAR, M.D.; JOSEPH GAFNI, M.D.; JORAM HELLER, M.D.
Arch Intern Med. 1961;107(4):539-550. doi:10.1001/archinte.1961.03620040065007.
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Renal disease occurs frequently in familial Mediterranean fever (F.M.F.) and is of grave prognostic significance. Mamou and Cattan, the first to recognize this, published detailed case histories and noted the familial pattern of occurrence.1 In 1952 we diagnosed amyloidosis as the cause of nephrotic syndrome in one of our patients, and autopsy confirmed the diagnosis. Since other cases of F.M.F. with signs of renal disease were already under observation, we considered the possibility of an inherent relationship between F.M.F. and amyloidosis.2,3 Since then, all autopsied cases of F.M.F. reported in the literature have disclosed amyloidosis,4-6 further strengthening this possibility. If we consider that in F.M.F. amyloidosis appears, most unusually and almost uniquely, as a part of a genetic disorder,7-9 it seems important to report our experience in a series of cases in various stages of renal disease who have been observed over many years.

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